Biomedical subjects

A Serraf

Publications and source records attributed to A Serraf.

At least 73 records · Page 4Linked to original sources

PubMed1996

Aortico-left ventricular tunnel in fetuses and infants.

BACKGROUND: Aortico-left ventricular tunnel is a rare congenital abnormal communication between the aorta and the left ventricle presenting in early childhood as aortic regurgitation and cardiac failure. This condition has rarely been reported in fetuses. Operation is the only treatment, and postoperative aortic incompetence could be related to the age or the type of repair. METHODS: We conducted a retrospective, two-institution review, from 1983 to 1995, of aortico-left ventricular tunnel diagnosed in utero and before 6 months of age. RESULTS: Three cases of aortico-left ventricular tunnel were diagnosed in utero by Doppler echocardiography between 22 and 24 weeks' gestation. Prenatal aortico-left ventricular tunnel was associated with severe left ventricular dysfunction, aortic valve anomalies, and fetal hydrops. One death occurred in utero and one immediately after birth, and in 1 case pregnancy was interrupted. In these 3 cases the diagnosis was confirmed by autopsy. Three neonates and 2 infants had the diagnosis of aortico-left ventricular tunnel made after birth and underwent successful surgical repair. At short and midterm follow-up all patients are alive and aortic valve regurgitation is absent or trivial. CONCLUSIONS: This series shows that aortico-left ventricular tunnel covers an anatomic spectrum of lesions. Cases diagnosed in utero by Doppler echocardiography are characterized by severe ventricular dysfunction, associated aortic valve lesions, and poor outcome. Postnatal cases represent the more favorable end of the spectrum, with no associated lesions, and can be repaired without mortality and with good functional results.

Adult↗

PubMed1996

Absent pulmonary valve syndrome: surgical treatment and considerations.

BACKGROUND: The operative management of absent pulmonary valve syndrome remains controversial regarding the need for pulmonary valve implantation and remodeling of pulmonary arteries. Moreover, symptomatic infants are considered to have a poor prognosis. This retrospective report summarizes the experience of a single institution. METHODS: Between May 1977 and May 1995, 37 consecutive patients underwent repair of absent pulmonary valve syndrome. Patients were divided into two groups according to age at operation: group A (10 infants less than 1 year old) and group B (27 patients older than 1 year). Mean age at operation was 5 +/- 4 months in group A and 72 +/- 42 months in group B. Initially, repair consisted of ventricular septal defect closure and relief of right ventricular outflow tract obstruction combined with pulmonary valve implantation. More recently, the concept of treatment has evolved with pulmonary arterioplasty without pulmonary valve insertion, except in patients with elevated pulmonary artery pressure. RESULTS: Of the 37 patients, 34 had successful repair. The overall in-hospital mortality rate was 8% (two deaths in group A and one in group B). No hemodynamic data were correlated with operative death. Death was associated with longer extracorporeal circulation time (p = 0.005) and longer aortic cross-clamping time (p = 0.019). In fact, these were clearly related to more complex anatomy (p = 0.001): multiple ventricular septal defects in 1, left pulmonary artery arising from the ductus in another, and left pulmonary artery arising from the aorta in the remainder. Follow-up was available in 22 of the 34 survivors. Mean follow-up time was 30 +/- 47 months in group A and 38 +/- 33 months in group B. All but 1 had no restriction of exercise, and most of them had pulmonary incompetence of Doppler echocardiography. One developed severe exercise intolerance because of pulmonary valve stenosis (xenograft), leading to uneventful reoperation 123 months after initial repair. One infant died suddenly of complete atrioventricular block 3 months after repair. The late mortality rate was 5%. CONCLUSIONS: Surgical treatment of absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. This procedure is feasible and is recommended especially in markedly symptomatic infants.

Case-Control Studies↗

PubMed1996

Surgical treatment of subaortic stenosis after biventricular repair of double-outlet right ventricle.

Out of 180 patients who underwent biventricular repair of double-outlet right ventricle between 1980 and 1995, 9 (5%) required reoperation because of subaortic stenosis. Two other patients who initially underwent operation elsewhere underwent reoperation at our institution because of subaortic stenosis. The median age at biventricular repair was 4 months. Repair consisted of tunnel construction from the left ventricle to the aorta in nine patients; the remaining two patients received an arterial switch operation with ventricular septal defect closure. Subaortic stenosis developed with time: the mean postoperative left ventricle-to-aorta gradient after repair was 10 +/- 19 mm Hg (range, 0 to 50 mm Hg) and became 84 +/- 27 mm Hg (range, 40 to 124 mm Hg) in a mean delay of 45 +/- 66 months (range, 1 to 213 months). At reoperation, the obstruction was caused by the protrusion of the inferior rim of the ventricular septal defect into the left ventricular outflow tract associated with subaortic hypertrophied muscle and membrane. The 11 patients underwent 15 reoperations. Surgical technique consisted of an extended septoplasty in 6 reoperations. In this technique an incision was made in the septal patch and was extended into the muscle toward the apex until a large opening of the left ventricular outflow pathway was obtained. A new patch was then secured to streamline the left ventricular outflow tract. None of the patients who underwent extended septoplasty had to undergo reoperation. There were no early or late deaths. At 115 +/- 85 months after biventricular repair, all patients were in New York Heart Association functional class I or II and the mean postoperative left ventricle-to-aorta gradient was 20 +/- 24 mm Hg (range, 0 to 60 mm Hg). We conclude that after biventricular repair of double-outlet right ventricle, the subaortic region is at risk for the development of stenosis. Surgical treatment adapted to the anatomy of the obstruction can offer good early and midterm results. It seems that an aggressive approach by an extended septoplasty avoids multiple reoperations.

Adolescent↗

PubMed1996

Repair of interrupted aortic arch: a ten-year experience.

Eighty-two consecutive patients with interrupted aortic arch were referred to our institution between 1985 and 1995. Three died before any attempt at operation and 79 underwent surgical repair. Median age at operation was 9 days (range 1 day to 6 years) and median weight was 3.0 kg (range 1.8 to 20 kg). All but one were in severe congestive heart failure and 31.5% had oliguria or anuria. Preoperative pH varied between 6.8 and 7.4 (median 7.3). Sixty-nine received prostaglandin E1 infusion and 54 received mechanical ventilation. Aggressive preoperative ressucitation was necessary in 43 cases. Preoperative transfontanellar echography (performed routinely) since 1987 revealed intracerebral bleeding in six patients. Type A interrupted aortic arch was present in 37 cases, 41 patients had type B, and one had type C. Interrupted aortic arch was associated with single ventricular septal defect in 35 cases, 24 patients had associated complex heart defects, and 30 had significant subaortic stenosis (six had both subaortic stenosis and complex association). Aortopulmonary window was found in four patients, truncus arteriosus was found in eight, and transposition of the great arteries was found in five, double-outlet right ventricle was found in one, single ventricle was found in three, multiple ventricular septal defects were found in two and superior-inferior ventricles were found in one. Sixty-four patients underwent single-stage repair and 15 underwent multistage repair. Aortic arch repair consisted of direct anastomosis in 59 cases, patch augmentation in eight, and conduit interposition in 12. Ten patients underwent associated pulmonary artery banding and 19 underwent concomitant repair of complex associated lesions. The subaortic stenosis was addressed by four surgical techniques: myotomy or myectomy in five patients; creation of a double-outlet left ventricle, aortopulmonary anastomosis, and conduit insertion between the right ventricle and pulmonary artery bifurcation in four; no direct attempt to relieve the subaortic stenosis in six; and left-sided ventricular septal defect patch in 15. Mean duration of deep hypothermic circulatory arrest, crossclamp time, and cardiopulmonary bypass time were 38.8 +/- 15.6 min, 60.5 +/- 24.7 min, and 143 +/- 40.1 min, respectively. Postoperative mortality rate was 18.9% (70% confidence limits 14% to 24.6%), and overall mortality rate was 31% (70% confidence limits 20.9% to 42.2%). The results have improved with time, with an overall operative mortality rate of 12% since 1990. Univariate statistical analysis revealed that early survival was influenced by preoperative renal function, detection of cerebral bleeding by transfontanellar echography, the number of cardioplegic injections, and the date of operation. Multivariate analysis revealed that preoperative renal function and the number of cardioplegic injections were independent risk factors for early mortality. Echocardiographic measurements of the left heart-aorta complex with preoperative Z values as low as-4 demonstrated rapid growth after repair. In the presence of subaortic stenosis, better survival was obtained with a left-sided patch for ventricular septal defect closure (p < 0.05). Twenty-three patients underwent 26 reoperations for recoarctations (seven), left bronchial compression (two), second-stage repair (eight), right ventricle-pulmonary artery conduit replacement (three), and miscellaneous (four). One of the survivors was reoperated on for subaortic membrane. Survival at 5 years for the entire series was 70%. For isolated forms, it was 73.5% (90% for 1990 to 1995), for complex forms it was 70%, and in the presence of subaortic stenosis it was 60%. In conclusion, interrupted aortic arch remains a surgical challenge with continually improving results. Early diagnosis with preoperative resuscitation and adequate myocardial protection seem extremely important for further improvements. Associated subaortic stenosis or complex lesions

Aorta↗

PubMed1996

Unusual forms of tracheobronchial compression in infants with congenital heart disease.

Three groups of unusual forms of tracheobronchial compression caused by vascular anomalies are presented. Three patients had an encircling right aortic arch with a left-sided descending aorta and ligamentum arteriosum (group 1), two patients had airway compression caused by a pincer effect between a malposed and enlarged ascending aorta and the descending aorta (group 2), and three patients had airway compression after an arterial switch operation for transposition of the great arteries (group 3). Symptoms developed in all patients before the age of 4 months, and six of them had multiple failed attempts at extubation before the surgical intervention directed at relieving the airway compression. Fiberoptic endoscopy was used in all patients as a first-line diagnostic tool and was 100% accurate in establishing the diagnosis. The operations performed were aortic uncrossing in group 1, dissection and aortopexy of the right or left main bronchus in group 2, and dissection of the left main bronchus and lysis of adhesions in group 3. In group 1 there was one early death, resulting from aspiration, and one late death 4 years later, resulting from an unrelated cause. In this group, bronchomalacia was noted after the operation and resolved gradually in the year after the intervention. In group 2, one patient died of an aortobronchial fistula after placement of a bronchial stent. Group 3 patients had good postoperative results. Two of them are completely symptom-free and one has residual bronchomalacia and may need placement of a bronchial stent.

Aorta↗

PubMed1996

Surgical approaches for double-outlet right ventricle or transposition of the great arteries associated with straddling atrioventricular valves.

The surgical management of patients with double-outlet right ventricle or transposition of the great arteries and straddling atrioventricular valves remains a subject of controversy. Biventricular repair has theoretic advantages because it establishes normal anatomy and physiology. In some instances, however, it seems to carry too high operative risk, and a univentricular heart repair is preferred. Since 1984, we have operated on 34 patients with double-outlet right ventricle (n = 15) or transposition of the great arteries (n = 19) with isolated straddling tricuspid valve (n = 17), isolated straddling mitral valve (n = 9), both mitral and tricuspid straddling (n = 2), or abnormal insertion of tricuspid (n = 7) or mitral (n = 2) chordae in the left ventricular outlet, precluding an adequate tunnel construction. Straddling was categorized according to the location of the papillary muscle insertion in the opposite ventricular chamber: type A, on the edge of the ventricular septal defect (n = 14); type B, on the opposite side of the ventricular septum away from the edge of the defect (n = 8); type C, on the free wall of the opposite ventricular chamber (n = 8). Abnormal chordal insertions were classified according to the location of their attachments around the edges of the defect. Three types of chordal distribution were identified: on the aortic conus, on the pulmonary conus crossing the ventricular septal defect, or around the defect closing it like a curtain. All but three patients had two ventricles of adequate size. Sixteen patients underwent palliation. Median age at the definitive operation was 6.5 months (range 1 to 130 months). Thirty patients underwent a biventricular repair and four had a univentricular repair. Biventricular repair was achieved by an arterial switch operation in 18 patients and by tunnel construction from the left ventricle to the aorta in 12. In isolated straddling of types A and B, the ventricular septal defect was closed by adjusting the septal patch on the ventricular side above the straddled papillary muscle. In type C, the patch was sewn over the papillary muscle by applying it on the septum. In double straddling, the ventricular septum was incised between the two papillary muscles, and an ellipsoid patch was used to reconstruct the septal defect, directing each subvalvular apparatus into its own ventricular chamber. When the abnormal chordae in the left outflow tract inserted on the aortic or pulmonary conus, the conus was incised and tailored to make a flap, leaving an unobstructed left ventricular outflow tract. In two patients the subvalvular apparatus was resected and reattached to the patch. Curtainlike chordae were a contraindication to biventricular repair in double-outlet right ventricle but not in transposition. There were four early deaths and one late death, all occurring in the group having biventricular repair. Death was due to myocardial ischemia (n = 1), right ventricular hypoplasia (n = 1), pulmonary hypertension (n = 1), and residual subaortic stenosis (n = 1). Two patients had moderate to severe postoperative atrioventricular valve incompetence, caused by a cleft in the mitral valve in one patient. Three patients were reoperated on for subaortic stenosis (n = 1), pulmonary stenosis (n = 1), and mitral regurgitation (n = 1). Mean follow-up of 30.7 +/- 19.4 months was achieved in the survivors. All but one patient (univentricular repair) were in New York Heart Association class I, without atrioventricular valve incompetence. Actuarial survival at 4 years was 85.3% +/- 3%. We conclude that straddling or abnormal distribution of chordae tendineae of the atrioventricular valves does not preclude biventricular repair in double-outlet right ventricle or transposition of the great arteries provided that the ventricles are of adequate size. Curtainlike abnormal tricuspid chordae remain a contraindication to biventricular repair in double-outlet right ventricle.

Cardiac Surgical Procedures↗

PubMed1996

Interleukin-10 release related to cardiopulmonary bypass in infants undergoing cardiac operations.

To evaluate cytokine balance related to cardiopulmonary bypass, we prospectively investigated 11 infants undergoing cardiac operations for congenital heart disease. Proinflammatory cytokines (tumor necrosis factor-alpha and interleukin-8) and the antiinflammatory cytokine interleukin-10 were measured at multiple time points before, during, and after bypass. Tumor necrosis factor-alpha and interleukin-8 values were within normal range before the operation. These values increased significantly during bypass, reaching their peaks after protamine administration (tumor necrosis factor-alpha, 133.6 +/- 124.9 pg/ml; mean +/- standard deviation; p<0.005) and 2 hours after termination of the procedure (interleukin-8, 92.1 +/- 44.1 pg/ml; p < 0.01). Tumor necrosis factor-alpha and interleukin-8 equaled normal prebypass values from the first postoperative day on. Interleukin-10 levels were within normal range before the operation and were already significantly increased 10 minutes after initiation of bypass (interleukin 10, 39.4 +/- 34.3 pg/ml; p<0.05). These levels remained elevated throughout the procedure but returned to normal after protamine administration. A second significant release of interleukin-10 occurred from the early postoperative period on, reaching its peak 24 hours after termination of cardiopulmonary bypass (interleukin-10, 351.6 +/- 304.0 pg/ml; p < 0.01). Interleukin-10 values were normal on the second postoperative day in all patients. Interleukin-10 kinetics showed an inverse pattern compared with tumor necrosis factor-alpha and interleukin-8. This difference suggests an interplay between proinflammatory and antiinflammatory cytokines released during and after cardiopulmonary bypass. Interleukin-10 levels measured 4 and 24 hours after bypass strongly correlated with the degree of hypothermia during bypass (Spearman's correlation coefficient, -0.77 [p < 0.01] and -0.89 [p < 0.0005], respectively); these levels did not correlate with duration of bypass and aortic crossclamping, however. This result suggests that besides immunologically mediated production of interleukin-10, hypothermia itself could modulate interleukin-10 production. In conclusion, this study demonstrates interleukin-10 production, in addition to interleukin-8 and tumor necrosis factor-alpha synthesis, in response to cardiopulmonary bypass in infants. Interleukin-10 could play a protective role by down-regulating proinflammatory cytokine release during and after cardiopulmonary bypass.

Anesthesia, General↗

PubMed1996

Truncus arteriosus repair: influence of techniques of right ventricular outflow tract reconstruction.

Fifty-six consecutive patients underwent total correction of truncus arteriosus. Median age at repair was 41 days, with a range of 2 days to 8 months. In 71% the operation was done in the first 2 months of life. Nine patients had complex forms of truncus and 11 patients had aortic insufficiency. The truncal aortic root was transected, which provides a clear exposure of the coronary ostia. The aorta was reconstructed by direct end-to-end anastomosis, and the truncal valve was preserved in every case. Several different techniques were used for pulmonary reconstruction, including three types of anatomic reconstruction of the pulmonary valve with a trisigmoid leaflet system and two types of nonanatomic reconstruction. The anatomic techniques included use of 33 Dacron valved conduits, eight homograft valved conduits, and one porcine aortic root bioprosthesis. The nonanatomic reconstructions included direct anastomosis to the right ventricle in nine patients and insertion of autologous pericardial valved conduits in five. The hospital mortality was 16% (9/56; 95% confidence limits, 2% to 30%). Multivariate analysis outlines two independent incremental risk factors for hospital death: nonanatomic pulmonary valve reconstruction techniques and age younger than 1 month. The hospital mortality was 7.1% in the group with anatomic pulmonary valve reconstruction versus 43% in the group with nonanatomic pulmonary valve reconstruction (p = 0.015). The hospital mortality was 5.7% in those older than 1 month versus 33% in those younger than 1 month of age (p = 0.04). There were two late deaths. The actuarial freedom from reoperation and angioplasty at 7 years was 100% for patients receiving pericardial conduits, 80% for those undergoing direct anastomosis, 77% for those receiving Dacron conduits, and only 43% for those receiving homografts (p = 0.02). In conclusion, anatomic reconstruction of the pulmonary valve seems important at the time of the operation, age younger than 1 month remains an incremental risk factor, and the truncal valve can be preserved.

Age Factors↗

PubMed1996

[Surgical treatment of isolated multiple ventricular septal defects. apropos of a series of 175 consecutive cases].

Between January 1980 and September 1995, 175 children were operated for closure of isolated multiple ventricular septal defects (VSD) (mean age 20 +/- 8 months, mean weight 8.1 +/- 4.4 kg). Eighty-nine patients had pulmonary protection: pulmonary artery banding (n = 76) and pulmonary valvular stenosis (n = 13). The remainder had severe pulmonary hypertension (mean pulmonary systolic pressures: 75.7 +/- 20.5 mmHg). The surgical strategy was based on the site of the VSD and the ventricular dominance determined preoperatively or at surgery. The VSD were perimembranous in 141 cases, muscular in 175 cases, of the inlet septum in 19 cases and infundibular in 13 cases. A surgical approach through a right atriotomy was adequate for complete repair in 122 patients; a second approach was necessary by right (n = 35) or left ventriculotomy (n = 4) or both (n = 2) or via the pulmonary artery in 2 cases. The operative mortality was 7.4% (13 patients). The causes of death were: residual (VSD (n = 6), pulmonary hypertension (n = 2), ventricular hypoplasia (n = 2), myocardial infarction (n = 3). Of the 162 survivors, 33 had residual VSDs, 12 were reoperated once (n = 11) or twice (n = 1). The mortality was 54.5%. The patients were classified in two groups: Group I (n = 130), those operated before 1990, and Group II (n = 45), the ones operated afterwards. The low muscular VSDs and left ventriculotomy were risk factors for mortality in univariate analysis for Group I and the "Swiss Cheese" type for Group II.

Cardiac Surgical Procedures↗

PubMed1995

Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Risk factors related to stage I mortality.

BACKGROUND: We reviewed 212 consecutive patients who underwent stage I palliative surgery for hypoplastic left heart syndrome (HLHS) at our institution between January 1983 and June 1993. METHODS AND RESULTS: Six surgeons participated in the care of these patients. Follow-up is 97% complete. Preoperative anatomic and physiological factors and procedural features of the stage I operation were analyzed for impact on stage I mortality, survival to stage II palliation, and actuarial survival. Hospital mortality was not significantly lower during the second half of the study period (P = .242). Operative mortality was 46.2%. Multivariate analysis revealed improved stage I operative survival in patients with mitral stenosis (MS) and aortic stenosis (AS; P = .006). Additional risk factors for stage I mortality were a lower immediately pre-stage I pH (P = .034) and weight < 3 kg (P = .015). Overall first-year actuarial survival for MS/AS was 59%, and it was 33% for all others (P = .001). Among stage I survivors, patients with MS/AS were more likely to survive to stage II palliation (P = .031). Analysis of actuarial survival of stage I survivors showed that a smaller ascending aorta (P < .001), aortic atresia (P < .001), and mitral atresia (P = .002) were all risk factors for intermediate death. CONCLUSIONS: Preoperative anatomic and physiological state are predictors of stage I mortality. HLHS anatomic subtype also influences intermediate outcome, most notably pre-stage II attrition. These data may be useful in choosing initial management for patients with HLHS.

Analysis of Variance↗

PubMed1995

[Right posterolateral thoracotomy in the treatment of of simple and isolated lesions of the heart].

The right posterolateral thoracotomy approach was used in 13 young girls aged from 1 to 8 years for open heart repair of atrial septal defect (ostium secundum and primum) and ventricular septal defect, without morbidity nor mortality. Neither the thoracotomy scar nor impaired growth of the mammary gland were visible. This approach is considered as safe as median sternotomy for open heart repair of isolated and simple cardiac defects, in prepuberty young females.

Child↗

PubMed1995

Endothelial dysfunction in venous pulmonary hypertension in the neonatal piglet.

In a group of neonatal piglets an increase in pulmonary arterial pressure was obtained within 2 weeks after a partial mechanical obstruction of the left atrium by a balloon catheter. Mean pulmonary artery pressure in the hypertensive animals (n = 6) was 24 +/- 2 mm Hg as compared (p < 0.01) with 15 +/- 1 mm Hg in controls (n = 6) or 9 +/- 2 mm Hg in sham-operated piglets (n = 6). Cardiac index was reduced in hypertensive versus control and sham groups: 0.15 +/- 0.01 versus 0.32 +/- 0.05 and 0.29 +/- 0.04 L.min-1.kg-1 (p < 0.05), respectively. There was no detectable difference on histologic examination in the pulmonary arteries between the three groups. Right ventricular hypertrophy was observed in the group with pulmonary hypertension. In hypertensive piglets, isolated conduit pulmonary arteries did not relax when stimulated with acetylcholine; they always relaxed to sodium nitroprusside. These data suggest that the first stages of perturbations reported during pulmonary venous hypertension occur at the level of the pulmonary vascular endothelium. This neonatal model of pulmonary hypertension is simple to perform and might be useful for further investigations.

Acetylcholine↗

PubMed1995

Systemic obstruction in univentricular hearts: surgical options for neonates.

BACKGROUND: The surgical management for bridging patients with univentricular heart and systemic obstruction to a Fontan procedure remains controversial. METHODS: Twenty-seven of 96 patients with univentricular heart and unobstructed pulmonary blood flow referred for surgical palliation were seen with systemic obstruction. Twenty-six were neonates with coarctation of the aorta in 21 and subaortic stenosis in 5. In 8 other patients, subaortic stenosis developed after initial pulmonary artery banding. Four different palliative procedures were performed: coarctation repair with pulmonary artery banding (group I, n = 15); Norwood or Damus-Kaye-Stansel or arterial switch operation (group II, n = 9); coarctation repair with pulmonary artery banding and bulboventricular foramen enlargement (group III, n = 2); and orthotopic heart transplantation with coarctation repair (group IV, n = 1). RESULTS: The mortality rate was 34.3% (n = 12) for all patients, 53.3% in group I, 33.3% in group II (p = 0.003 versus group I), and 50% in group III. Nine patients (8 in group I and 1 in group II) had development of subaortic stenosis and underwent a subsequent procedure: Damus-Kaye-Stansel operation in 5, arterial switch operation in 3, and bulboventricular foramen enlargement in 1. Three had a concomitant or subsequent Fontan procedure and 2, a bidirectional Glenn procedure. In group II, 1 patient underwent a subsequent Fontan procedure and another, a bidirectional Glenn anastomosis. Six of the 8 patients with subaortic stenosis after initial pulmonary artery banding underwent a second stage consisting of a Damus-Kaye-Stansel procedure (n = 3), bulboventricular foramen enlargement (n = 2), or creation of an aortopulmonary window (n = 1). Three had a concomitant Fontan procedure and 2, a bidirectional Glenn procedure. Actuarial 4-year survival was 65.5% +/- 8.4% (70% confidence limits) for all patients; it was 40% +/- 13.3% in group I and 66.6% +/- 16.3% in group II (p < 0.05). CONCLUSIONS: Initial management of patients with univentricular heart and systemic obstruction by Norwood-like procedures provides a better outcome. Success of the Fontan operation relies on the ability to provide timely relief of subaortic stenosis.

Aortic Coarctation↗

PubMed1995

Reoperation after the arterial switch operation for transposition of the great arteries.

Although most children after an arterial switch operation for transposition of the great arteries have normal development and cardiac function, a few require reoperation. During the last 10 years, 68 of 753 patients who underwent arterial switch operations (9.3%) underwent 75 reoperations. Thirty underwent early reoperation (< 30 days or during the same hospital stay) and 38 underwent late reoperation. Causes for reoperation included pacemaker insertion (n = 5), left diaphragm plication (n = 4), revision for hemostasis (n = 1), mediastinitis (n = 2), superior vena cava thrombosis (n = 9), subvalvular pulmonic stenosis (n = 5), supravalvular pulmonic stenosis (n = 16), residual atrial (n = 2) or ventricular (n = 8) septal defects, isolated mitral valve insufficiency (n = 2), aortic valve insufficiency (either isolated [n = 1] or in association with mitral incompetence [n = 1] or stenosis [n = 1]), left coronary artery ostial stenosis (n = 1), and recurrent aortic (n = 6) or neoaortic (n = 4) aortic coarctation. In all but 27 patients, the residual defects were already present immediately after the completion of the arterial switch operation; however, only patients with critical lesions were reoperated on early. Interventional catheterization procedures were performed when indicated; however, they only postponed inevitable reoperation. Successful relief of superior vena cava thrombosis was achieved by atriojugular bypass grafting in two patients, by early open thrombectomy in six patients, and by direct patch angioplasty of the superior vena cava once. Patch plasty for subvalvular or supravalvular pulmonic stenosis was carried out in 21 patients, septal defect closure was carried out in nine patients, and pulmonary artery banding was performed in one patient with criss-cross atrioventricular relationship and multiple ventricular septal defects. Valve repair was performed in all five patients with either isolated or combined aortic and mitral valve dysfunction. One patient with left coronary ostial stenosis underwent a patch enlargement of this ostium. Recoarctation was repaired by end-to-end anastomosis in eight patients and by a subclavian flap and a patch angioplasty in one patient each. Seven patients underwent a second reoperation for supravalvular pulmonary stenosis (n = 3), mitral valve replacement (n = 1), ventricular septal defect closure (n = 1), and recurrent coarctation (n = 2). There were six intraoperative (8.8%) and two late deaths. All early deaths occurred after early reoperations.(ABSTRACT TRUNCATED AT 400 WORDS)

Aortic Coarctation↗

PubMed1995

Transposition of the great arteries [S,D,L]. Pathologic anatomy, diagnosis, and surgical management of a newly recognized complex.

The transposition of the great arteries [S,D,L] complex is delineated for the first time from the anatomic, diagnostic, and surgical standpoints in this study of 26 cases: 16 surgical and 10 postmortem. Transposition of the great arteries with situs solitus of the viscera and atria (S), D-loop ventricles (D), and L-transposition (L) was characterized by six additional interrelated anomalies that largely determined surgical management: (1) ventricular septal defect, usually conoventricular, in 96%; (2) malalignment of the conal septum, typically leftward and posteriorly, in 80%; (3) right ventricular hypoplasia in 50%; (4) pulmonary outflow tract stenosis in 27%; (5) ventricular malposition, such as superoinferior ventricles, in 23%; and (6) absent left coronary ostium resulting in "single" right coronary artery in 23%. Complete surgical repair was done in 81% of the surgical patients with a 12.5% hospital mortality rate and no late deaths. When there was no pulmonary outflow tract stenosis and intracardiac anatomy was uncomplicated, we undertook anatomic repair before 1 month of age. However, when pulmonary outflow tract stenosis coexisted, complete repair was deferred until after age 1 year, our currently preferred operation being the REV procedure (réparation a l'etage ventriculaire). When complex intracardiac anatomy precluded biventricular repair, a palliative procedure was performed in 19% without mortality. Hence, this experience indicates that surgical management of patients with the transposition of the great arteries [S,D,L] complex is feasible.

Child↗

PubMed1995

Surgical management of neonatal coarctation.

Between 1983 and 1994, 307 consecutive neonates underwent coarctation repair by a single surgical technique: extended end-to-end anastomosis. Mean age at operation was 13 +/- 8 days. Isolated coarctation was present in 95 patients (group 1), 102 patients had associated ventricular septal defect (group 2), and 110 patients had associated complex intracardiac lesions (group 3). Aortic arch hypoplasia was present in 81% of the patients (62% in group 1 versus 85% in group 2 and 93% in group 3: p < 0.001). In 271 patients, the aortic arch reconstruction was performed via a left thoracotomy with normothermia (100% of group 1, 95% of group 2, and 72% of group 3); in the other 36 patients, undergoing one-stage repair or palliation of the associated lesion, it was performed via a midline sternotomy during a short period of deep hypothermia and circulatory arrest (5% of group 2 and 28% of group 3). Pulmonary artery banding was performed in 94 patients. Spontaneous ventricular septal defect closure was observed in 39% of the patients of group 2 operated on via thoracotomy. Early mortality rates in groups 1 (2%) and 2 (2%) were significantly lower than in group 3 (17%) (p < 0.001). There were 29 late deaths, all related to associated cardiac lesions or their subsequent repair. The overall total mortality was 16.9%. In group 3 this rate was significantly higher in patients undergoing two-stage procedures (47%) than in those undergoing one-stage repair (23%) (p < 0.05). All but 14 survivors were followed up for a mean of 61 +/- 36 months. Actuarial survivals at 10 years were 98% in group 1, 94% in group 2, and 60% in group 3. The recoarctation rate was 9.8%, leading to 21 reoperations and three angioplasties without mortality. Patients with a more extended or severe form of aortic arch hypoplasia had a significantly higher risk of recoarctation (p < 0.001). Actuarial freedom from reoperation for recoarctation at 10 years was 93%. The findings of this study suggest that extended end-to-end anastomosis provides an adequate and safe repair of neonatal coarctation. Low recoarctation rate, owing to effective relief of the obstruction created by aortic arch hypoplasia and to complete resection of ductal tissue, freedom from major morbidity, and feasibility via both lateral and anterior approaches are the main advantages of the extended end-to-end anastomosis.(ABSTRACT TRUNCATED AT 400 WORDS)

Aortic Coarctation↗

PubMed1995

Surgery for congenital mitral valve disease in the first year of life.

Between 1980 and 1993, 20 patients less than 1 year of age underwent operations for congenital mitral valve disease. Ten patients had congenital mitral incompetence and 10 had congenital mitral stenosis. Mean age was 6.6 +/- 3.4 months and mean weight was 5.6 +/- 1.5 kg. Atrioventricular canal defects, univentricular heart, class III/IV hypoplastic left heart syndrome, discordant atrioventricular and ventriculoarterial connections, and acquired mitral valve disease were excluded. Indications for operation were intractable heart failure or severe pulmonary hypertension, or both. Associated lesions, present in 90% of the patients, had been corrected by a previous operation in seven. In congenital mitral incompetence there was normal leaflet motion (n = 3), leaflet prolapse (n = 2), and restricted leaflet motion (n = 5). In congenital mitral stenosis anatomic abnormalities were parachute mitral valve (n = 4), typical mitral stenosis (n = 3), hammock mitral valve (n = 2), and supramitral ring (n = 1). Mitral valve repair was initially performed in 19 patients and valve replacement in one with hammock valve. Concurrent repair of associated lesions was performed in 12 patients. The operative mortality rate was zero. There were six early reoperations in five patients for mitral valve replacement (n = 4), a second repair (n = 1), and prosthetic valve thrombectomy (n = 1). One late death occurred 9 months after valve replacement. Late reoperations for mitral valve replacement (n = 2), aortic valve replacement (n = 1), mitral valve repair (n = 2), subaortic stenosis resection (n = 1), and second mitral valve replacement (n = 1) were performed in five patients. Actuarial freedom from reoperation is 58.0% +/- 11.3% (70% confidence limits 46.9% to 68.9%) at 7 years. After a mean follow-up of 67.6 +/- 42.8 months, 94% of living patients are in New York Heart Association class I. Doppler echocardiographic studies among the 13 patients with a native mitral valve show mitral incompetence of greater than moderate degree in one patient and no significant residual mitral stenosis. Overall, six patients have mitral prosthetic valves with a mean transprosthetic gradient of 6.2 +/- 3.7 mm Hg. These results show that surgical treatment for congenital mitral valve disease in the first year of life can be performed with low mortality. Valve repair is a realistic goal in about 70% of patients and possibly more with increased experience. Reoperation rate is still high and is related to complexity of mitral lesions and associated anomalies, but late functional results are encouraging.

Follow-Up Studies↗

PubMed1995

Surgery for tetralogy of Fallot at less than six months of age. Is palliation "old-fashioned"?

There is still no consensus regarding the most appropriate protocol for managing symptomatic patients with tetralogy of Fallot (TOF) presenting during early infancy. From January 1987 to April 1994, 83 patients with TOF were operated on at less than 6 months of age. Sixty patients (mean age 109 +/- 5 days) underwent primary repair and 23 (mean age 72 +/- 10 days) underwent initial palliation with a modified Blalock-Taussig shunt (MBTS) in 22 cases. No strict protocol was used but patients who received initial palliation had lower weight, smaller pulmonary annulus or had an anomalous coronary artery. Nineteen of the patients initially palliated underwent repair at a median of 13 months after palliation. During this interval, the pulmonary annulus size increased from a Z-value of -3.0 +/- 0.4 at palliation to -1.6 +/- 0.7 at repair (P = 0.06) and the summed diameters of pulmonary artery branches from -2.2 +/- 0.4 to -1.6 +/- 0.7 (P = 0.2). There were no operative deaths among the repaired patients, but two patients died early after shunt insertion. Transannular patch was required in 58% of the patients regardless of the management protocol. Actuarial survival rate and freedom from reoperation at 48 months were 98.0% and 77.4% (P = 0.003) and 87.6% and 90.0% in primarily repaired and initially palliated patients, respectively. Primary repair of TOF at the time of presentation is the preferred approach regardless of age.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗