Search PubMed⌕ Search

Biomedical subjects

R M Freedom

Publications and source records attributed to R M Freedom.

At least 91 records · Page 5Linked to original sources
PubMed1997

Azygos vein agenesis and PAPVR: a potential surgical hazard.

A 15-year-old boy underwent surgical correction of partial anomalous pulmonary venous return (right upper and middle lobe veins) into the high superior vena cava with an intact atrial septum using entirely autologous tissue. The rare association of azygos vein agenesis and partial anomalous pulmonary venous return should be emphasized to prevent the inadvertent inclusion of the anomalous systemic vein (hemiazygos vein) into the pulmonary venous circuit during the surgical repair.

Adolescent↗
PubMed1997

The modified Blalock-Taussig shunt: clinical impact and morbidity in Fallot's tetralogy in the current era.

BACKGROUND: The Blalock-Taussig shunt is considered a low-risk management option for palliation in tetralogy of Fallot, but the morbidity associated with a Blalock-Taussig shunt can have a significant impact on patient care. We reviewed the outcome for this operation in the current era. METHODS: Between 1990 and 1994, 65 children with tetralogy of Fallot received a modified Blalock-Taussig shunt. Sixty patients who had follow-up angiography were assessed for clinical outcomes and shunt-related morbidity and mortality. From the same study period, 68 of 247 pediatric patients who underwent angiography and tetralogy repair, but did not receive palliation, were randomly selected to comprise a comparison group. RESULTS: Palliation was more likely in the presence of a complicated tetralogy malformation or if there was an associated medical condition. Median age at palliation was 58 days (range: 1 to 535 days). Ninety-five percent of shunts were right-sided. Self-limited morbidity complicated 11% of shunt operations. Significantly smaller distal right pulmonary arteries were observed in the palliated group before total repair compared with findings in the group without palliation and 33% of patients who underwent palliation had angiographic evidence of pulmonary artery distortion. Shunt stenosis was common and correlated with younger age at palliation. Shunt occlusion resulted in one death. Excluding noncardiac causes of death, overall survival was 90% in the palliated group versus 97% in the nonpalliated group (p = 0.09). CONCLUSIONS: Pulmonary artery hypoplasia and angiographic evidence of pulmonary artery distortion are common after initial palliation by a modified Blalock-Taussig shunt. Neonatal palliation was associated with significantly smaller pulmonary arteries before repair, which necessitated additional interventions.

Anastomosis, Surgical↗
PubMed1997

Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience.

OBJECTIVES: Our purpose was to assess the risk factors for late mortality, loss of sinus rhythm and atrial flutter after the Mustard operation. BACKGROUND: The Mustard operation provides correction of cyanosis with low surgical risk in transposition of the great vessels. However, right ventricular failure, loss of sinus rhythm, atrial flutter and death are frequent long-term complications. METHODS: Records of 534 children who underwent the Mustard operation at a single center since 1962 were reviewed for demographic, anatomic, electrocardiographic and physiologic predictors and outcomes. RESULTS: There were 52 early deaths (9.7%). Survival analysis was undertaken for 478 early survivors with a mean follow-up interval of 11.6 +/- 7.2 years. There were 77 late deaths (16.1%), with sudden death (n = 31) the most frequent cause. Survival estimates were 89% at 5 years and 76% at 20 years of age. Risk factors were an earlier date of operation, operative period arrhythmia and an associated ventricular septal defect. Risk (hazard) of late death declined in the first decade, with further peaks in the second decade. Sinus rhythm was present in 77% at 5 years and 40% at 20 years. Loss of sinus rhythm was associated with previous septectomy, postoperative bradycardia and late atrial flutter. Freedom from atrial flutter was 92% at 5 years and 73% at 20 years of age. Risk factors for atrial flutter were the occurrence of perioperative bradyarrhythmia, reoperation and loss of sinus rhythm during follow-up. Risk of atrial flutter demonstrates a late increase. CONCLUSIONS: Ongoing loss of sinus rhythm and late peaks in the risk of atrial flutter and death necessitate continued follow-up.

Arrhythmias, Cardiac↗
PubMed1997

Balloon angioplasty of recurrent coarctation: a 12-year review.

OBJECTIVES: This study was undertaken to investigate the long-term outcome of balloon angioplasty for recurrent coarctation of the aorta in a large series of patients. BACKGROUND: Balloon angioplasty has become the standard treatment for residual or recurrent aortic coarctation. Despite the widespread use of this treatment modality, there are few data outlining the long-term outcome of a large patient cohort. METHODS: Clinical, echocardiographic, hemodynamic and angiographic data on 90 consecutive patients who underwent balloon angioplasty between January 1984 and January 1996 were reviewed. RESULTS: Mean systolic pressure gradients were reduced from 31 +/- 21 to 8 +/- 9 mm Hg after dilation (p = 0.0001). The mean diameter of the stenotic site, measured in the frontal and lateral views, increased by 38% and 35%, respectively (p = 0.001). Neurologic events occurred in two patients, with one death. An aortic tear occurred in one patient, requiring surgical intervention. Optimal results were defined as a postprocedure gradient < 20 mm Hg and were obtained acutely in 88% of patients. At long-term follow-up (12 years), 53 (72%) of 74 patients with an early optimal result remained free from reintervention. Transverse arch hypoplasia, defined as an arch dimension < 2 SD below the mean for age, was the primary predictor of the need for reintervention. CONCLUSIONS: Although the majority of patients undergoing percutaneous balloon angioplasty for recoarctation of the aorta will achieve long-term benefit, the need for further surgical intervention in those with transverse arch hypoplasia remains high.

Adolescent↗
PubMed1997

Balloon dilation of severe aortic stenosis in the neonate: comparison of anterograde and retrograde catheter approaches.

OBJECTIVES: We sought to compare anterograde and retrograde balloon dilation of severe aortic valve stenosis in neonates. BACKGROUND: There is a high incidence of iliofemoral artery complications after retrograde balloon dilation of the aortic valve in the neonate. Therefore, a nonarterial technique of catheter access to the aortic valve would be worth exploring. METHODS: Group 1 included 11 consecutive patients (median age 6 days, range 1 to 42; median weight 3.5 kg, range 2.16 to 4.25) undergoing attempted anterograde dilation through a femoral venous approach. Group 2 included 15 patients (median age 3 days, range 1 to 35; median weight 3.4 kg, range 2.5 to 4.4 kg) who underwent attempted retrograde dilation, including 2 in whom attempted anterograde approach had failed. RESULTS: The valve was successfully crossed in 9 of 11 anterograde and 13 of 15 retrograde dilations. In both groups, the peak gradient across the valve decreased significantly (both p = 0.001). On echocardiography, the jet width of the aortic incompetence/ annulus diameter ratio was 0.16 +/- 0.08 (mean +/- SD) after anterograde and 0.51 +/- 0.24 after retrograde dilation (p = 0.03), possibly because of unrecognized valve leaflet perforation. Two patients in group 1 developed persistent, mild mitral insufficiency. Femoral artery thrombosis developed in one patient after anterograde dilation and in eight after retrograde dilation (p = 0.03). CONCLUSIONS: This series demonstrates that an anterograde approach for balloon angioplasty of severe neonatal aortic valve stenosis is feasible, achieves good hemodynamic relief and lessens morbidity compared with retrograde arterial techniques.

Age Factors↗
PubMed1996

Echocardiographic assessment of pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect.

OBJECTIVES: The purpose of this study was to determine the role of high resolution two-dimensional echocardiographic imaging and color flow Doppler study in assessing the pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect (VSD). BACKGROUND: Although echocardiography is a well established technique for assessing central pulmonary arteries in pulmonary atresia VSD and for determining the presence or absence of a patent arterial duct, few data are available on its role in patients whose source of blood supply is from collateral vessels. METHODS: Forty-two patients aged a few hours to 19 months (mean 29 days) were prospectively assessed by high resolution echocardiography, including color flow Doppler study, during a 4-year period ending in 1994, before any intervention other than intravenous administration of prostaglandins. Angiographic confirmation was available in 29 patients, including 18 (95%) of 19 with aortopulmonary collateral channels. RESULTS: A patent arterial duct was correctly identified as the sole source of pulmonary blood supply in 23 patients, whereas aortopulmonary collateral channels were detected in 19, with one of these having a small patent arterial duct and collateral channels. The patent arterial duct originated from the undersurface of the aorta in 16 (67%) of 24 patients and from the base of the brachiocephalic trunk in 7 (33%) of 24. All patients with a patent ductus as the sole source of pulmonary blood supply had confluent pulmonary arteries. Nonconfluent pulmonary arteries were present in six patients, with all but one having aortopulmonary collateral channels as the sole source of pulmonary flow. Aortopulmonary collateral channels were direct in 17 (89%) of 19 patients, whereas in 2 (11%) of 19, both direct and indirect collateral channels were present. Color flow Doppler study was accurate in determining the presence or absence, the side and the origin of the collateral channels in all patients, with the correct number being determined in 12 (67%) of 18. "Wash-in" to the hilar pulmonary arteries (retrograde color flow) was seen in 12 (92%) of 13 patients with collateral channels and confluent pulmonary arteries. Failure to identify a tiny central pulmonary artery occurred in one patient. CONCLUSIONS: High resolution imaging and color flow Doppler study provide good appreciation of the source of pulmonary blood supply in neonates and young infants with pulmonary atresia VSD.

Angiography↗
PubMed1996

Aortic valve regurgitation after surgical versus percutaneous balloon valvotomy for congenital aortic valve stenosis.

To compare characteristics of aortic regurgitation (AR), the results of 213 procedures (110 balloon aortic valvotomies [BAV] and 103 surgical aortic valvotomies [SAV]) for treatment of congenital aortic valve stenosis were reviewed. These procedures were performed in 187 patients from June 1981 to September 1993. Echocardiograms recorded immediately before, within 6 months afterward, and at latest follow-up were compared. Color Doppler was used to assess the degree of AR and was quantified as the ratio of the regurgitant jet width to valve annulus, the jet width ratio. Whereas BAV patients were older (median age 5.7 years vs 3 months; p = 0.0001), there was no significant difference in median follow-up interval (3.1 years [range 0.5 to 7.2] for BAV vs 3.6 years [range 0.6 to 10.4] for SAV; p = 0.44). The mean balloon-to-annulus ratio for BAV was 0.99 +/- 0.09. An open valvotomy was performed in 83% of surgical cases. Acute systolic gradient reduction and subsequent increase at late follow-up was similar for both groups. Acutely, the mean jet width ratio increased similarly (p = 0.84) for BAV (+9 +/- 15%; p = 0.0001) and SAV (+9 +/- 12%; p = 0.0003) and was not related to age at procedure. At late follow-up, mean jet width ratio further increased significantly in both groups, although there was no difference (p = 0.17) in amount of progression (BAV +10 +/- 12%; p = 0.0001, SAV +15 +/- 13%; p = 0.0002). Thus, BAV and SAV produce AR of similar severity with similar rates of progression.

Aortic Valve↗
PubMed1996

Tetralogy of Fallot with anomalous LAD: repair without conduit.

We report a case of anomalous origin of left anterior descending coronary artery in a patient with tetralogy of Fallot, absent pulmonary valve cusps, and origin of left pulmonary artery from the ascending aorta. Pulmonary outflow tract obstruction was successfully treated by direct anastomosis of the main pulmonary artery to the right ventricle. This approach prevented injury to the anomalous coronary artery and avoided the use of an extracardiac conduit.

Anastomosis, Surgical↗
PubMed1996

Morphologic and hemodynamic consequences after percutaneous balloon valvotomy for neonatal pulmonary stenosis: medium-term follow-up.

OBJECTIVES: This study sought to evaluate ventricular and valvular morphologic changes, hemodynamic consequences and clinical outcomes of pulmonary balloon valvotomy performed in the neonatal period. BACKGROUND: Previous studies support percutaneous balloon valvotomy as the management option of choice for infants and children with valvular pulmonary stenosis. Less information is available to define the impact of valvotomy on the clinical course when performed in the neonatal period. METHODS: Patient records, catheterization data, cineangiograms and selected echocardiograms (initial and most recent studies) of 37 consecutive neonates undergoing attempted balloon dilation were reviewed. RESULTS: Dilation was accomplished in 35 (94%) of 37 attempts. Immediately after dilation, the transvalvular peak to peak systolic gradient decreased from 60 +/- 22 mm Hg (mean +/- SD, range 20 to 100) to 11 +/- 10 mm Hg (range 0 to 45) (p < 0.0001), and the right ventricular/aortic systolic pressure ratio decreased from 1.25 +/- 0.43 (range 0.5 to 2.6) to 0.66 +/- 0.22 (range 0.2 to 1) (p < 0.0001). Oxygen saturation measured by percutaneous oximetry increased from 80 +/- 7% to 92 +/- 4% (p < 0.0001). Three patients died (8%), and two required repeat balloon dilation. At the follow-up visit (median 31 months, range 6 months to 8 years), the estimated peak instantaneous Doppler gradient was 15 +/- 9 mm Hg (range 6 to 36). Thickening of valve leaflets, initially present in 93% of patients, was found in only 4%, and leaflet mobility improved in all. Hypoplasia of the right ventricle, initially present in 31%, was found in only 4% at the latest evaluation. Pulmonary annulus diameter Z score increased from -3 +/- 1.0 to 0 +/- 0.1 (p < 0.0001). Freedom from reintervention was 90%, 84% and 84% at 1, 2 and 8 years, respectively. CONCLUSIONS: These data support the application of balloon valvotomy as the initial intervention in the treatment algorithm for neonates with critical pulmonary valve stenosis. Medium-term follow-up observations demonstrate sustained hemodynamic relief and support maturation of the right ventricle and pulmonary valve annulus, with the expectation of a good long-term outcome.

Algorithms↗
PubMed1996

Superior vena cava to pulmonary artery anastomosis: an adjunct to biventricular repair.

From May 1981 to September 1995, 38 patients received a superior vena cava-pulmonary artery anastomosis in association with biventricular repair. Patients were divided into four groups on the basis of indication for operation. Group A (19 patients) had a small physiologic right ventricle defined by tricuspid anulus z values or predicted right ventricular volume. Group B (11 patients) had a functionally compromised right ventricle. Group C (four patients) consisted of those receiving a superior vena cava-pulmonary artery anastomosis as a facilitation to biventricular repair. Group D (four patients) was defined by acute postoperative right ventricular dysfunction. Age ranged from 5 months to 51 years (median 3.5 years). There were 14 different underlying primary diagnoses in this cohort and multiple associated anomalies. Operative mortality was as follows: group A, two of 19 (10.5%); group B, two of 11 (18%); group C, none of four (0%); and group D, three of four (75%). Follow-up is complete in 37 of 38 patients (97%), ranging from 1 to 174 months (mean 46.3 +/- 36.9). Twenty-two patients are in New York Heart Association functional class I and eight patients are in class II. No clinical evidence of cyanosis or protein-losing enteropathy has been detected. With the use of this adjunctive approach, acceptable intermediate-term outcomes were obtained in patients having an anatomically or functionally compromised pulmonary ventricle. The anastomosis safely facilitates repair in a subset of patients. Results for this procedure when used as a salvage operation for right ventricular dysfunction have not been satisfactory.

Adolescent↗
PubMed1996

Scimitar syndrome: twenty years' experience and results of repair.

BACKGROUND: Thirty-two patients with scimitar syndrome were seen in the period between 1975 and 1995. There were 11 male and 21 female patients. Median age at diagnosis was 7 months (mean 7.7 years, range 1 day to 70 years). Patients in whom the diagnosis was made during the first year of life (infantile group, n = 19) had more severe symptoms and had a higher incidence of heart failure (11/19 vs 0/13) and of pulmonary hypertension (11/19 vs 1/13) than did the patients in whom the diagnosis was made after age 1 year (adult group, n = 13). In 17 patients the anomalous pulmonary venous drainage was repaired by baffling the vein to the left atrium. The median age at this operation was 5.8 years (mean 14.8 years, range 6 months to 70 years). RESULTS: No deaths occurred in this surgical group during a mean follow-up period of 8.9 years (range 1.6 to 17 years). Eight patients (47%), however, had evidence of pulmonary venous stenosis after repair, and two required reoperation for pulmonary venous obstruction. All six children in the infantile group had postoperative pulmonary venous stenosis, compared with two of 11 older patients. Postoperative quantitative pulmonary perfusion scans performed in 15 patients demonstrated reduced flow to the right lung (24%, range 0% to 59%). CONCLUSION: We conclude that age at detection of scimitar syndrome is important in predicting outcome. Surgical repair seldom results in normal blood flow to the right lung but abolishes left-to-right shunt. Postoperative pulmonary venous obstruction is prevalent, especially in the infants.

Adolescent↗
PubMed1996

Augmentation of pulmonary blood flow with an axillary arteriovenous fistula after a cavopulmonary shunt.

Of 320 children with cyanotic congenital heart malformations who had previously undergone cavopulmonary shunt operations, 11 had increasing cyanosis and exercise intolerance and were considered unsuitable for definitive repair, a Fontan procedure, or other palliation. Eight had a previous Glenn shunt and three had a previous bidirectional cavopulmonary connection. To augment pulmonary blood flow, 10 patients underwent creation of an ipsilateral axillary arteriovenous fistula. Mean oxygen saturations were 80% +/- 2% before operation, 85% +/- 2% immediately after operation, and 84% +/- 3% at a mean follow-up interval of 7.4 years (range 0.1 to 15.5 years). Mean hemoglobin values were 202 +/- 10 gm/L before operation, 177 +/- 10 gm/L after operation, and 191 +/- 14 gm/L at latest review. The only complication was mild swelling of the arm distal to the fistula in one patient. All patients reported improvement in exercise tolerance. Eight patients have continued evidence of fistula patency. Development of ipsilateral pulmonary arteriovenous fistulas has not been observed in any patient. Creation of an axillary arteriovenous fistula to augment pulmonary blood flow after a cavopulmonary shunt provides useful palliation for complex cyanotic heart disease when other options are limited. Such additional sources of pulmonary blood flow may influence the development of pulmonary arteriovenous fistulas.

Arteriovenous Shunt, Surgical↗
PubMed1996

A symptomatic atypical mycobacterial infection masquerading as a vascular ring on chest x-ray.

Atypical mycobacterium is rare in children. There are few case reports of childhood pulmonary diseases caused by atypical mycobacterium. The case of a 20-month-old boy who had stridor and respiratory distress is presented. Chest x-ray showed right-sided narrowing of the trachea and left-sided aortic knob. Ziehl-Neelsen stain showed acid-fast bacilli. The culture showed Mycobacterium malmoense.

Angiography↗
PubMed1996

Massive right ventricular fibroma treated with partial resection and a cavopulmonary shunt.

Cardiac fibromas in infants and children may present as intramural or intracavitary masses. Total or partial resection as well as transplantation have been reported as treatment for symptomatic patients. We report an infant, diagnosed prenatally, who underwent partial excision of a massive obstructive right ventricular fibroma and creation of a bidirectional cavopulmonary shunt. The palliative approach has allowed the child to be followed up with the option of transplantation should the tumor continue to increase in size.

Female↗