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T L Spray

Publications and source records attributed to T L Spray.

At least 55 records · Page 3Linked to original sources
PubMed2000

Evaluation of ventricular septal defect repair using intraoperative transesophageal echocardiography: frequency and significance of residual defects in infants and children.

Intraoperative transesophageal echocardiography (IOTEE) is commonly used to assess for residual defect and the need to return to bypass after repair of ventricular septal defect (VSD). The frequency and significance of residual septal defects as noted on IOTEE has not been well defined. We evaluated the frequency of residual VSD via IOTEE and the relationship between size of a residual VSD and rate of reoperation. In addition, we looked at the relationship between the presence of a residual VSD via IOTEE and the presence of residual VSD at follow-up transthoracic echocardiography (TTE). Residual VSD was measured via the largest width of the Doppler color jet diameter originating at the left ventricular septal surface. Of the 294 patients evaluated with IOTEE after VSD repair, one-third had a residual defect by IOTEE Doppler color flow mapping. Two-thirds of these defects closed spontaneously on TTE by the time of hospital discharge. There was no difference in frequency of residual VSD between simple (VSD closure alone, n = 90) and complex (VSD with associated lesions, n = 204) repair. Return to bypass with immediate reoperation was undertaken in nine patients, all of whom had significant shunt via oximetry (Qp/Qs > 1.5:1.0). All had residual VSD color jet diameters > 3 mm. Seven patients had residual color jet equal to 3 mm; however, hemodynamic studies did not reveal a significant shunt and none of these had reoperation. Seven patients with no VSD or < 3 mm residual VSD via had late reoperation to close residual VSD at 4 days to 5 months after initial operation. These were due to patch dehiscence or development of an "intramural" VSD in patients with conotruncal anomaly. A residual defect on IOTEE color Doppler measuring > or = 4 mm predicts the need for immediate reoperation, while a 3 mm defect may be significant and requires additional intraoperative hemodynamic evaluation. The majority of small defects noted on IOTEE are not present at discharge TTE. Patients with conotruncal defect repair should be followed closely for development of late significant "intramural" defects.

Age Factors↗
PubMed2000

Assessment of pulmonary/systemic blood flow ratio after first-stage palliation for hypoplastic left heart syndrome: development of a new index with the use of doppler echocardiography.

OBJECTIVE: Circulatory maldistribution is believed to be a major cause of early death after first-stage palliation for hypoplastic left heart syndrome. Flow reversal in the reconstructed aorta may reflect the pulmonary/systemic blood flow ratio. The purpose of our study was to investigate the utility of arterial PO (2), arterial oxygen saturation, and a newly developed Doppler-derived flow index in predicting the pulmonary/systemic flow ratio after first-stage palliation for hypoplastic left heart syndrome. METHODS: Twenty-four infants who underwent first-stage palliation for hypoplastic left heart syndrome or a variant were studied. Superior vena cava blood samples were drawn to estimate the mixed venous saturation and permit calculation of the pulmonary/systemic blood flow ratio. Fifty-four samples were evaluated within the first 24 hours after surgery. Simultaneous blood draw and Doppler echocardiography were performed with interrogation in the distal aspect of the arch reconstruction. The ratio of the Doppler velocity-time integral of retrograde flow to the velocity-time integral of forward flow was calculated and compared with the pulmonary/systemic blood flow ratio RESULTS: The median mixed venous saturation for the 54 samples was low (38.5%; range, 18%-64%). The median calculated pulmonary/systemic blood flow ratio was 1.4:1 (range, 0.3:1 to 4. 2:1). Pulse pressure, mixed venous saturation, and arterial PO (2) were not statistically significant predictors of the measured pulmonary/systemic blood flow ratio. Although both aortic oxygen saturation (R (2) = 0.84, P <.01) and Doppler flow reversal ratio (R (2) = 0.94, P <.001) were significantly associated with the measured pulmonary/systemic blood flow ratio, the model coefficient of determination was greatest for Doppler flow reversal ratio. CONCLUSION: Measures of arterial oxygen saturation and arterial PO (2) may be misleading in assessing the circulatory status of infants after first-stage palliation for hypoplastic left heart syndrome. Doppler echocardiography, through use of the Doppler flow reversal ratio, provides a more useful measure of pulmonary/systemic blood flow ratio. Low mixed venous saturation after surgery may be due to factors other than pulmonary overcirculation, such as ventricular dysfunction and low cardiac output.

Blood Flow Velocity↗
PubMed2000

Modifications to the cavopulmonary anastomosis do not eliminate early sinus node dysfunction.

OBJECTIVE: To determine whether operations that theoretically jeopardize the sinus node (hemi-Fontan and/or lateral tunnel Fontan procedures) are associated with a greater risk of sinus node dysfunction than those that theoretically spare the sinus node (bidirectional Glenn and/or extracardiac conduit). METHODS: Between January 1, 1996, and December 31, 1999, a prospective cohort study was conducted evaluating the incidence of sinus node dysfunction in patients undergoing a bidirectional Glenn or hemi-Fontan procedure and those in whom the Fontan repair was completed with either an extracardiac conduit or a lateral tunnel. Sinus node dysfunction was defined (1) as a heart rate more than 2 SD below age-adjusted norms or (2) as a predominant junctional rhythm and/or a sinus pause of more than 3 seconds as determined by the resting electrocardiogram and/or ambulatory monitoring at hospital discharge. RESULTS: Fifty-one patients had a bidirectional Glenn shunt (mean age 7.8 +/- 5.1 months) and 79 a hemi-Fontan procedure (mean age 6.9 +/- 2.8 months). The incidence of sinus node dysfunction on postoperative day 1 was significantly higher after the hemi-Fontan (36%) than after the bidirectional Glenn shunt (9.8%); however, by hospital discharge this difference was no longer apparent (hemi-Fontan [8%]; bidirectional Glenn [6%]; P = not significant). No difference in early sinus node dysfunction was discernible after the extracardiac conduit (4/30 [13%]) compared with the lateral tunnel Fontan procedure (6/46 [13%]) (P = not significant). No diagnostic or perioperative variables were predictive of sinus node dysfunction. CONCLUSIONS: Avoidance of surgery near the sinus node has no discernible effect on the development of early sinus node dysfunction. Thus, concerns about early sinus node dysfunction should not override patient anatomy or surgeon preference as determinants of which cavopulmonary anastomosis to perform.

Anastomosis, Surgical↗
PubMed2000

Necrotizing enterocolitis in neonates with congenital heart disease: risk factors and outcomes.

OBJECTIVE: Necrotizing enterocolitis (NEC) is primarily a disease of the premature infant. Among children born at term, however, congenital heart disease may be an important predisposing factor for this condition. To determine risk factors for NEC in patients with congenital heart disease, we conducted a case-control study of neonates with cardiac disease admitted to the cardiac intensive care unit at our center during the 4-year period from January 1995 to December 1998. METHODS: Cardiac diagnosis and age at admission were analyzed for association with NEC among the 643-patient inception cohort. Demographic, preoperative, and operative variables were recorded retrospectively in 21 neonates with congenital heart disease who developed NEC and 70 control neonates matched by diagnosis and age at admission. Using parametric and nonparametric analysis, cases and controls were compared with respect to previously identified risk factors for NEC. RESULTS: Among the entire cohort of 643 neonates with heart disease admitted to the cardiac intensive care unit, diagnoses of hypoplastic left heart syndrome (odds ratio [OR] = 3.8 [1.6-9.1]) and truncus arteriosus or aortopulmonary window (OR = 6.3 [1.7-23.6]) were independently associated with development of NEC by multivariable analysis. In the case-control analysis, earlier gestational age at birth (36.7 +/- 2. 7 weeks vs 38.1 +/- 2.3 weeks), prematurity (OR = 3.9 [1.2-12.5]), highest dose of prostaglandin >0.05 microg/kg/minute (OR = 3.9 [1. 2-12.5]), and episodes of low cardiac output (meeting specific laboratory criteria) or clinical shock (OR = 6.5 [1.8-23.5]) correlated with the development of NEC. Earlier gestational age and episodes of low output were the only factors that remained significantly associated with NEC by multivariable analysis. Although there was no difference in hospital mortality between patients with and without NEC, mean hospital stay was significantly longer in those who developed NEC (36 +/- 22 days vs 19 +/- 14 days). CONCLUSIONS: The risk of NEC in neonates with congenital heart disease is substantial. Factors associated with an elevated risk of NEC in infants with heart disease include premature birth, hypoplastic left heart syndrome, truncus arteriosus, and episodes of poor systemic perfusion or shock. Heightened suspicion is warranted in newborns with these risk factors.

Case-Control Studies↗
PubMed1999

Early results of the Ross procedure in simple and complex left heart disease.

BACKGROUND: The Ross procedure has been used increasingly to treat aortic valve disease in children and young adults. Benefits include the lack of anticoagulation after surgery and the potential growth and durability of the autograft. The purpose of this study was to review our institutional experience with the Ross procedure and to compare early outcome in simple aortic valve disease and complex left heart disease. METHODS AND RESULTS: Between January 1995 and October 1998, 66 patients (median age, 10.8 years; range, 6 days to 34.8 years) underwent the Ross procedure. The primary indication for surgery was isolated valvular disease in 41 patients: aortic stenosis (AS; n=3), aortic insufficiency (AI; n=11), and AS/AI (n=27). The remaining 25 patients had multiple levels of left ventricular outflow tract obstruction, 12 of whom had at least moderate AI. Additional left heart disease in the complex group included subaortic stenosis (n=20), arch obstruction (n=7), mitral valve disease (n=5), apical aortic conduit stenosis or insufficiency (n=3), and supravalvar AS (n=2). There were 123 prior interventions performed in 51 patients, including aortic valvotomy/valvuloplasty (n=56), coarctation repair (n=21), subaortic stenosis resection/Konno procedure (n=10), ventricular septal defect closure (n=8), apical aortic conduit placement (n=3), aortic valve replacement (n=3), and other (n=22). An isolated Ross procedure was performed in 41 patients, 10 of whom required concurrent aortic annulus enlargement procedure to accommodate the larger pulmonary autograft. In the remaining 25 patients, 49 concurrent procedures were performed, including the Konno procedure (n=17), aortic annulus enlargement (n=2), subaortic membrane resection (n=9), arch augmentation (n=5), mitral valvuloplasty (n=5), ventricular septal defect closure (n=4), apicoaortic conduit division (n=3), and other (n=4). One patient (1.5%) died 3 days after a Ross-Konno procedure, which included arch reconstruction, from presumed arrhythmia. There were no other early deaths. One patient required ECMO (extracorporeal membrane oxygenation) for 3 days after a ventricular tachycardia (VT)-related cardiac arrest. Transient complete heart block was seen in 4 patients; the duration was <5 days. No patient had left ventricular outflow tract obstruction on discharge echocardiography. Neo-AI was graded as none (n=5), trivial-mild (n=57), or moderate (n=3). All 3 patients with moderate neo-AI at discharge had abnormal pulmonary valves before surgery. Perioperative VT was noted in 18 patients (27.2%), 2 of whom were discharged on antiarrhythmic medication. CONCLUSIONS: The Ross procedure can be performed in isolation or in combination with other complex procedures with low mortality (1.5%) and acceptable short-term results, even in patients with complex left heart disease and multiple prior interventions. Postoperative VT is common. Anatomic abnormalities of the pulmonary valve preclude its use as an autograft.

Adolescent↗
PubMed1999

Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome.

BACKGROUND: Results of staged palliation for hypoplastic left heart syndrome (HLHS) have improved in recent years; however, certain risk factors have been associated with decreased survival rates. METHODS AND RESULTS: We retrospectively reviewed the medical records of 67 patients weighing </=2.5 kg undergoing the first stage of reconstructive surgery at our institution between January 1, 1990, and December 31, 1997. HLHS was present in 45 patients, complex double-outlet right ventricle in 10, unbalanced AV canal in 5, tricuspid atresia with transposition of the great vessels in 4, and other diagnoses in 3. Mean age at surgery was 10.1+/-10.7 days (median, 8 days), and mean weight was 2.2+/-0.3 kg (median, 2.2 kg). Fourteen patients weighed </=2.0 kg, and 2 patients weighed </=1.5 kg. Early mortality (death within 30 days or before hospital discharge) was 51% (34 of 67). No patient, procedural, or time-related variables correlated with increased mortality. However, there was a trend toward increased mortality with increased cardiopulmonary bypass time (P=0.076) and decreased preoperative ventricular performance (P=0.139). CONCLUSIONS: These findings suggest that low weight alone in a patient with HLHS or an anatomic variant should not be considered a contraindication to staged reconstructive surgery.

Body Weight↗
PubMed1999

Caval contribution to flow in the branch pulmonary arteries of Fontan patients with a novel application of magnetic resonance presaturation pulse.

BACKGROUND: A complete understanding of fluid mechanics in Fontan physiology includes knowledge of the caval contributions to right (RPA) and left (LPA) pulmonary arterial blood flow, total systemic venous return, and relative blood flow to each lung. METHODS AND RESULTS: Ten Fontan patients underwent cine MRI. Three cine scans of the pulmonary arteries were performed: (1) no presaturation pulse, (2) a presaturation pulse labeling inferior vena cava (IVC) blood (signal void), and (3) a presaturation pulse labeling superior vena cava (SVC) blood. The relative signal decrease is proportional to the amount of blood originating from the labeled vena cava. This method was validated in a phantom. Whereas 60+/-6% of SVC blood flowed into the RPA, 67+/-12% of IVC blood flowed toward the LPA. Of the blood in the LPA and RPA, 48+/-14% and 31+/-17%, respectively, came from the IVC. IVC blood contributed 40+/-16% to total systemic venous return. The distributions of blood to each lung were nearly equal (RPA/LPA blood=0.94+/-11). CONCLUSIONS: In Fontan patients with total cavopulmonary connection, SVC blood is directed toward the RPA and IVC blood is directed toward the LPA. Although the right lung volume is larger than the left, an equal amount of blood flow went to both lungs. LPA blood is composed of equal amounts of IVC and SVC blood because IVC contribution to total systemic venous return is smaller than that of the SVC. This technique and these findings can help to evaluate design changes of the systemic venous pathway to improve Fontan hemodynamics.

Blood Flow Velocity↗
PubMed1999

Usefulness of intraoperative transesophageal echocardiography in predicting the degree of mitral regurgitation secondary to atrioventricular defect in children.

The objectives of this study were to determine the validity of the grade of mitral regurgitation (MR) as imaged by intraoperative transesophageal echocardiography (TEE) in predicting the grade of MR at follow-up. Intraoperative TEE and corresponding follow-up transthoracic studies were retrospectively reviewed and the regurgitant jet area to left atrial area ratio was used to quantify the MR. Patient records were reviewed to identify factors contributing to the development of a certain grade of MR. Intraoperative TEE was useful in detecting severe MR that required further repair at the same time. However, discrepancy in the grade of MR at follow-up was noted in 47% of patients (21 of 47) and unchanged grade of MR was found only in 53% of patients (26 of 47). Blood pressures were significantly lower and heart rates higher intraoperatively. Initial preoperative grade of MR and type of atrioventricular canal defect did not predispose for a particular grade of MR at follow-up. The grade of MR by intraoperative TEE does not predict the grade of MR at follow-up as imaged by transthoracic echocardiography.

Adolescent↗
PubMed1999

Successful use of alternate waste nitrogen agents and hemodialysis in a patient with hyperammonemic coma after heart-lung transplantation.

BACKGROUND: Lethal hyperammonemic coma has been reported in 2 adults after lung transplantation. It was associated with a massive elevation of brain glutamine levels, while plasma glutamine levels were normal or only slightly elevated. In liver tissue, glutamine synthetase activity was markedly reduced, and the histologic findings resembled those of Reye syndrome. The adequacy of therapy commonly used for inherited disorders of the urea cycle has not been adequately evaluated in patients with this form of secondary hyperammonemia. OBJECTIVE: To determine whether hemodialysis, in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy, would be efficacious in a patient with hyperammonemic coma after solid-organ transplantation. DESIGN: Case report. SETTING: A children's hospital. PATIENT: A 41-year-old woman with congenital heart disease developed a hyperammonemic coma with brain edema 19 days after undergoing a combined heart and lung transplantation. METHODS: Ammonium was measured in plasma. Amino acids were quantitated in plasma and cerebrospinal fluid by column chromatography. The effectiveness of therapy was assessed by measuring plasma ammonium levels and intracranial pressure and performing sequential neurological examinations. RESULTS: The patient had the anomalous combination of increased cerebrospinal fluid and decreased plasma glutamine levels. To our knowledge, she is the first patient with this complication after solid-organ transplantation to survive after combined therapy with sodium phenylacetate, sodium benzoate, arginine hydrochloride, and hemodialysis. Complications of the acute coma included focal motor seizures, which were controlled with carbamazepine, and difficulty with short-term memory. CONCLUSIONS: The aggressive use of hemodialysis in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy may allow survival in patients after solid-organ transplantation. An acute acquired derangement in extra-central nervous system glutamine metabolism may play a role in the production of hyperammonemia in this illness that resembles Reye syndrome, and, as in other hyperammonemic disorders, the duration and degree of elevation of brain glutamine levels may be the important determining factors in responsiveness to therapy.

Adult↗
PubMed1999

Heart/single-lung transplant for a "failed Fontan" with pulmonary A-V malformation.

Pulmonary arteriovenous malformation can occur in patients with functional single ventricle after a cavopulmonary connection. There is no effective medical treatment for this complication. Pulmonary arteriovenous malformations may regress over time after heart transplantation, but may be a source of significant perioperative morbidity. We report the case of a woman with single ventricle, ventricular dysfunction, and bilateral pulmonary arteriovenous malformations who had successful treatment of both cardiac and pulmonary failure with en bloc heart and right lung transplantation.

Adult↗
PubMed1999

Management of exsanguinating hemoptysis during cardiopulmonary bypass.

BACKGROUND: Large-volume hemoptysis during cardiopulmonary bypass is an infrequent, but life-threatening event. Rapid airway clearance and control are the primary prerequisites for successful management. METHODS: The cases of 3 patients with different sources of exsanguinating hemoptysis during cardiopulmonary bypass managed initially with rigid bronchoscopy were reviewed. RESULTS: In all patients, airway control was rapidly established and weaning from cardiopulmonary bypass CPB was accomplished. Two patients survived the operative procedure. The other patient died in the operating room of unremitting bilateral pulmonary hemorrhage. CONCLUSIONS: Major hemoptysis during cardiopulmonary bypass is best dealt with initially by rapid airway control and cessation of bypass in an expeditious manner. An algorithm for suggested management is provided. The rigid bronchoscope is the optimal tool for initial management and it should always be available. Definitive treatment is determined by the cause and the persistence of hemorrhage once these maneuvers have been performed.

Aged↗
PubMed1999

Outcome after repair of tetralogy of Fallot with absent pulmonary valve.

BACKGROUND: Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is associated with pulmonary artery dilatation and airway compression. METHODS: Since January 1, 1984, 28 patients with TOF/ APV have undergone complete repair (median age 11 days, range 1 day to 16 years). RESULTS: Thirteen patients were ventilated for respiratory failure preoperatively and extracorporeal membrane oxygenation was used in 3. Twenty-six patients underwent pulmonary artery plication (11 anterior, 15 anterior/ posterior). The right ventricular outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (5), or monocusp valve (4). Early mortality was 21.4% (6/28), with 1 late death. All early deaths occurred in infants intubated preoperatively. Survival was 77% (95% confidence limit [CL] 56%, 89%) at 1 year and 72% (95% CL 50%, 86%) at 10 years. After surgery, 3 patients underwent reoperation for persistent respiratory symptoms, which resolved after repeat plication and placement of a valved conduit. Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year and 52% (95% CL 29%, 71%) at 10 years. In a multivariable analysis, only preoperative intubation was associated with a worse outcome (p = 0.04). CONCLUSIONS: Long-term outcome for patients with TOF/APV who survive the initial repair is good. Repeat plication and pulmonary valve implantation may improve outcome in patients with persistent airway compression.

Adolescent↗
PubMed1999

Comparison of left and right atrial epicardial pacing in patients with congenital heart disease.

BACKGROUND: Complex congenital heart disease (CHD) often necessitates the use of epicardial pacing. Adequate right atrial (RA) sensing and pacing thresholds are often difficult to obtain due to suture line scarring and RA dilatation. The purpose of this study was to evaluate the placement of left atrial (LA) epicardial leads in children. METHODS: Patient demographics, pacing, and sensing data of atrial pacing systems implanted between January 1994 and January 1997 were collected. RESULTS: Forty-nine pacing systems were implanted: 14 LA epicardial, 19 RA epicardial, and 16 transvenous in the right atrium. Lead impedance, current, and energy were similar in the two epicardial groups throughout the study. Energy thresholds (ET) were lower in the LA than RA at 6 months, and 1 and 2 years (p < 0.05). Analysis of post-Fontan patients performed alone revealed a lower ET in the LA as compared with the RA. Pacing and sensing parameters from transvenous leads are presented for relative comparison. CONCLUSIONS: Transvenous leads are most efficient but often contraindicated in complex CHD. LA leads offer lower energy thresholds than RA leads with similar sensing parameters.

Adolescent↗
PubMed1999

Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection.

BACKGROUND AND METHODS: Between January 1, 1984, and December 1, 1997, 73 infants with functional single ventricle and total anomalous pulmonary venous connection were admitted to our institution. A retrospective review was undertaken to determine factors influencing survival. RESULTS: Heterotaxy syndrome was present in 52 patients and hypoplastic left heart syndrome in 14. Obstructed total anomalous pulmonary venous connection was present in 21 patients. The pulmonary venous connection was supracardiac in 32 patients, cardiac in 21 patients, infracardiac in 13, and mixed in 7. Twelve patients died before the operation. The remaining 61 patients underwent surgery at a median age of 5 days (range 1 day-2. 5 years). Overall survival was 45% at 6 months of age, 37% at 1 year, and 19% at 5 years. Survival for patients undergoing surgery was 54% at 6 months of age, 44% at 1 year, and 23% at 5 years. By univariate analysis with the Cox proportional hazards model, younger age at the time of the initial operation and repair of total anomalous pulmonary venous connection were predictors of mortality. Lung tissue from 14 patients was available for histologic examination. The pulmonary veins were dilated and wall thickness was increased. Increased muscularization of the arteries was seen in 11 patients. CONCLUSIONS: The long-term prognosis for children undergoing staged reconstructive operations for single ventricle and total anomalous pulmonary venous connection is poor. Early mortality is high and late death is a continuing risk. Development of the pulmonary vasculature, especially the pulmonary veins, is abnormal, even in children without clinical evidence of pulmonary venous obstruction.

Cardiac Surgical Procedures↗
PubMed1999

The hypoplastic left heart syndrome with intact atrial septum: atrial morphology, pulmonary vascular histopathology and outcome.

OBJECTIVES: The purpose of this study was to investigate the outcome in infants with hypoplastic left heart syndrome and intact atrial septum and to evaluate the relationship of atrial morphology, left atrial decompression pathway and lung histopathology to outcome. BACKGROUND: In the hypoplastic left heart syndrome, severe restriction at the atrial level results in marked systemic hypoxemia after birth. Infants with intact atrial septum may be at high risk for mortality after Norwood operation. METHODS: Of 316 infants with hypoplastic left heart syndrome seen at our center over a 6.5-year period, 18 (5.7%) had intact atrial septum. Medical records and echocardiograms were reviewed. RESULTS: On echocardiography, three types of intact atrial septal morphology were identified: 1) large left atrium, thick prominent septum secondary with thin septum primary adherent (type A, n = 12); 2) small left atrium with thick, muscular atrial septum (type B, n = 4), and 3) giant left atrium, thin atrial septum with severe mitral regurgitation (type C, n = 2). Seven infants had left atrial decompression pathways that were severely obstructed (3/12 type A, 4/4 type B). Norwood operation was performed in 17 infants; one underwent emergency balloon atrial septostomy and died. Of six early survivors, all with type A atrial morphology and unobstructed decompression pathway, three died after subsequent cavopulmonary surgery. Lung histopathology revealed severely dilated lymphatics and "arterialization" of the pulmonary veins in those with the severest degree of obstruction to left atrial egress (type B atrial morphology). CONCLUSIONS: Despite aggressive intervention, outcome for infants born with hypoplastic left heart syndrome and intact atrial septum is poor. Maldevelopment of the pulmonary vasculature contributes to the high mortality seen. Atrial morphology can be used as a marker for the severity of pulmonary vascular disease.

Heart Atria↗
PubMed1999

Outcome of infants listed for lung or heart/lung transplantation.

BACKGROUND: Little is known about outcome, characteristics, or organ availability for infants listed for lung or heart/lung transplantation. METHODS: Within a 45-month period at one institution, all pediatric patients who were listed for primary lung or heart/lung transplantation and who reached the end point of either transplant or death prior to transplant were identified. Outcomes for those patients listed as younger than and older than 1 year of age were compared. RESULTS: Among 48 pediatric patients, 19 were infants less than one year of age. The median age among infants at listing was 3.7 months (range 0.5 to 8.9 months). Death before transplant occurred in 10 of 19 infants (53%) compared with 14 of 29 (48%) children. When comparing those infants who died prior to transplant with those who received organs, there were no significant differences with respect to size, blood type, age at listing, presence of pulmonary hypertension, or type of transplant for which the patient was listed. There was a trend toward poorer pre-transplant survival for infants when compared with children. Waiting times were significantly shorter for infants vs children (p = 0.02). The incidence of acute cellular rejection and serious infection was similar in the 2 groups. Infants had significantly longer hospitalization post-transplant and a trend toward poorer hospital survival, although survival at 1 year was comparable between the 2 groups. CONCLUSION: The outcome for infants listed for lung or heart/lung transplantation is similar to that of children; thus, very young age should not be considered a contraindication to lung or heart/lung transplantation. Earlier diagnosis and listing may decrease pre-transplant mortality.

Adolescent↗
PubMed1999

Vascular anomalies causing symptomatic tracheobronchial compression.

OBJECTIVES/HYPOTHESIS: To review the clinical presentation and diagnostic evaluation of patients with symptomatic congenital vascular anomalies causing tracheobronchial compression and to establish the short- and long-term results of surgical intervention with respect to postoperative complications, persistent symptoms, and ventilator and tracheostomy dependence. STUDY DESIGN: Retrospective review. METHODS: Chart review and telephone follow-up. RESULTS: Between 1987 and 1996, 35 children underwent surgical intervention to relieve symptomatic tracheobronchial compression resulting from a congenital vascular anomaly. Historically, the onset of symptoms occurs within the first months of life; however, only 12 (34%) of patients were diagnosed by 6 months of age and 13 (37%) were diagnosed at greater than 1 year of age. Excluding anomalous innominate artery, chest radiography or barium swallow was suggestive of a congenital vascular anomaly in 30 (94%) of the patients. Magnetic resonance imaging correctly delineated the anatomy of the vascular anomaly in 29 patients. Bronchoscopy was diagnostic in all three patients with anomalous innominate arteries causing tracheal compression. Postoperative follow-up was obtained in 32 (91%) of patients; 25 (78%) of these were asymptomatic at the time of their most recent examination. The remaining patients had persistent stridor, recurrent respiratory tract infections, and/or chronic cough. In all three patients who underwent postoperative bronchoscopy for persistent symptoms, tracheomalacia was demonstrated in the region of previous compression. CONCLUSIONS: Tracheobronchial compression from congenital vascular anomalies is a rare but treatable cause of respiratory symptoms. Early diagnosis requires a prompt, thorough clinical and radiologic evaluation. Surgery affords excellent long-term resolution of symptoms.

Adolescent↗