Lung perfusion scans in patients with congenital heart defects.

In 63 patients with various congenital heart defects, lung perfusion was evaluated with technetium-99mm macroaggregated albumin. Right lung perfusion abnormalities were documented in 34 patients (54%). A particularly high incidence occurred in patients who had undergone a systemic to pulmonary artery shunt operation as an initial palliative procedure or who had had right ventricular outflow reconstruction and in those with bilateral pulmonary artery stenosis. Serial studies were helpful in evaluating the functional results of different transcatheter interventions for optimizing pulmonary blood flow. The quantitative relative perfusion radionuclide method was a more sensitive means of detecting cases of abnormal lung perfusion than was chest radiology.

Steady state serum digoxin concentration in relation to digitalis toxicity in neonates and infants.

Steady state serum digoxin concentrations were determined in 34 neonates and infants receiving standard maintenance doses of the drug. Digitalis intoxication, diagnosed by ECG criteria, occurred in four of 13 patients with a serum concentration above 2 ng/ml and not in any of 21 subjects with a serum digoxin concentration below this level. This association was found to be significant. It seems that the concept of increased tolerance to digoxin hitherto ascribed to infants is not tenable and that the monitoring of serum digoxin concentration is essential to treatment in this age group.

Correction of tetrad of Fallot with reduced incidence of right bundle branch block.

In 20 patients who underwent a modified surgical repair of tetrad of Fallot complete right bundle branch block developed in only 8 (40 percent). Standard and intraoperative conduction studies indicated that in these patients the right bundle branch block was due to injury of the right bundle branch near the ventricular septal defect (proximal right bundle branch block). The modified operative technique is aimed at minimizing the injury to the right ventricle and it includes a significantly shorter than usual ventriculotomy incision and avoidance of the septal (moderator) band during infundibulectomy. Intra- and postoperative hemodynamic studies of these patients revealed that relief of the right ventricular outflow obstruction was optimal.

Aortico-left ventricular tunnel: collective review.

Aortico-left ventricular tunnel (ALVT) is an extremely rare congenital entity in which an abnormal communication between the aorta and the left ventricle bypasses the aortic valve. The condition usually results in gross aortic regurgitation, rapid cardiac decompensation, and death. An additional case of ALVT managed by successful surgical repair is described. The 38 cases reported in the literature are reviewed with emphasis on the clinical and pathological features, diagnosis, natural history, and management. Early operation is recommended to prevent distortion of the aortic valve, dilatation of the left ventricle, and distortion of the aortic anulus.

Ectopic origin of a coronary artery from the aorta. Sudden death in 3 of 23 patients.

Ectopic origin of a coronary artery from the aorta is uncommon. In the last decade, its importance as a possible cause of sudden death has been documented. Initially, only the left coronary artery was implicated. Lately, the ectopically arising right coronary artery has also been shown to be involved in cases of sudden death. We reviewed the pathologic anatomy in 23 cases of ectopic origin of a coronary artery from the aorta. In three of these cases, death could be attributed to ectopic origin of a coronary artery. In one case the left coronary artery arose from the right aortic sinus, and in two cases the right coronary artery arose from the left aortic sinus. In one of these, a scar of healed myocardial infarction was present in the inferior wall of the left ventricle. The possible mechanism for sudden death in these cases is reviewed and emphasis placed on the theory that the acute angle at which the ectopic artery leaves the aorta results in a flap-like mechanism at the arterial ostium. Ostial stenosis by the flap could be a significant factor in causing myocardial ischemia in some patients and also in sudden death.

Coarctation of the aorta with congenital hemangioma of the face and neck and aneurysm or dilatation of a subclavian or innominate artery. A new syndrome?

Congenital cutaneous hemangioma associated with congenital heart disease is extremely rare. We report four infants with congenital cavernous hemangioma of the face and neck and coarctation of the aorta. Three also had congenital aneurysm of a subclavian or innominate artery. One patient also had mild congenital valvular aortic stenosis. The unusual combination of lesions may represent a new syndrome.

Surgical management of ventricular septal defect with aortic valve prolapse: clinical considerations and results.

Aortic valve prolapse is found in over 5% of children with ventricular septal defect (VSD). Although this association occurs mostly with doubly committed subarterial VSDs, in this study the predominant type of VSD was perimembranous. In order to determine the need and timing for surgery and whether the anatomical features of septal defect may influence clinical management and outcome in this lesion, we reviewed our experience with 28 consecutive patients, operated on for VSD with prolapsed aortic valve cusp, with or without aortic regurgitation. Twenty-two patients had a perimembranous VSD and six had doubly committed VSD. Aortic regurgitation was trivial or absent in nine patients, mild in ten and moderate to severe in nine. Associated cardiac anomalies were present in 18 patients, all having perimembranous VSD, and included right ventricular outflow tract (RVOT) obstruction (n = 6), discrete subaortic membrane (n = 4) or both (n = 8). None of these patients had more than moderate aortic regurgitation. The patients underwent surgical closure of the septal defect between the ages of 1.5 and 34 years of age (median = 7). Sixteen patients having mild or trivial aortic regurgitation underwent closure of the VSD only, and 12 patients underwent VSD closure with aortic valvuloplasty. Valvuloplasty was required more often in doubly committed VSDs (66%) and in the perimembranous type without associated anomalies (100%), and significantly less often in the presence of RVOT obstruction, subaortic membrane or both (22%). At follow-up (up to 5 years, mean 18 months), the grade of aortic regurgitation was unchanged in 11 and decreased in 5 patients undergoing closure of the VSD only.(ABSTRACT TRUNCATED AT 250 WORDS)

Bidirectional flow in congenital ventricular septal defect: a Doppler echocardiographic study.

The purpose of this study was to demonstrate the value of combined two-dimensional and pulsed Doppler echocardiography (echo) in localizing and recording bidirectional flow in congenital ventricular septal defect. Eight children, aged 8 months to 16 years, with clinical signs of a ventricular septal defect, underwent two-dimensional and pulsed Doppler echo study prior to cardiac catheterization. The ventricular septal defect was documented anatomically by two-dimensional echo in all eight patients. Flow patterns in systole and diastole through the ventricular septal defect and on both sides of the defect were carefully studied. In all eight children, systolic, high velocity, pathologic, left to right flow was documented when the sampling volume was positioned on the right ventricular side of the defect. When the sampling volume was positioned inside the defect, to and fro flow, left to right in systole and right to left in diastole, was observed. In children with moderate to large defects, the diastolic flow had a peak in early diastole. Increased pressure in the right ventricle over the left ventricle during the same period was demonstrated by cardiac catheterization and coincided with the Doppler flow. The direction of flow across the defect was affected by the size of the defect and the magnitude of the net shunt. Two-dimensional and pulsed echo Doppler were shown to be useful in demonstrating the ventricular septal defect and estimating its size and hemodynamic significance noninvasively.

Wide vascular pedicle on thoracic roentgenogram in complete transposition of the great arteries.

A narrow vascular pedicle on the thoracic roentgenogram is an important sign in the clinical diagnosis of complete transposition of the great arteries (Shaher, 1973a). Rarely a wide vascular pedicle is present in this condition, mainly due to associated lesions. The differential diagnosis in these cases is discussed, based on our experience with 14 cases.

Hypothermia and exsanguination in emergency cardiovascular surgery in children.

Emergency cardiac surgery in children can be successfully carried out even when cardiopulmonary bypass is either not feasible or not available quickly enough. Hypothermia induced with ice-water bags, complete circulatory arrest, near-total body exsanguination, and rewarming with a heating blanket and heating lamps, were used twice successfully in the repair of a ruptured aneurysm of the ascending aorta.

Prosthetic valve replacement in infants and children.

A review of 41 children from 10 months to 16 years of age who had a valve replacement between the years 1966 to 1981 is reported. Sixty-one per cent of the valve deformities were rheumatic and 39% congenital. Twenty-two children had the mitral valve replaced, 14 had an aortic valve and 5 had both aortic and mitral valve replacement. There was a hospital mortality of 9.7% and only one later death during a mean follow up period of 6.75 years. Three children have required a second mitral valve replacement. Thrombo-embolic episodes were encountered in 4 children. The special problems of valve replacement in infants and children are discussed.

Cerebral arteriovenous fistula causing congestive heart failure in infancy.

The case of a newborn female in congestive heart failure due to a cerebral A-V fistula is presented. The cardiac manifestations in the presence of this condition are described, and the diagnostic importance of auscultation of the skull is stressed. Because of the precarious condition of most of these neonates, diagnostic procedures should be minimized if the clinical picture is compatible with this diagnosis, and cerebral angiography should be performed before any heart studies are undertaken.

Interruption of the aortic arch with complete transposition of the great arteries. Clinical and angiocardiographic diagnosis at the age of one day.

The association of complete transportation of the great arteries and complete interruption of the aortic arch is very rare. This combined lesion was diagnosed clinically in a 1-day-old infant in whom it caused cyanosis of the upper half of the body. The diagnosis was confirmed angiocardiographically. We believe this is the youngest patient in whom this diagnosis was made in vivo.

Successful pregnancy in a woman with cyanotic congenital heart disease after a palliative pulmonary-systemic shunt. A case report.

A 20-year-old woman with cyanotic congenital heart disease composed of corrected transposition of the great vessels, severe pulmonic stenosis, atresia of the left pulmonary artery and a large ventricular septal defect, had a successful pregnancy following a pulmonary-systemic shunt (Blalock-Taussig). The hemoglobin decreased from 21 to 16 g/dL following the operation. The antepartum course was complicated by intrauterine growth retardation and pregnancy-induced hypertension. A normal fetal nonstress test and biophysical profile permitted continuation of the pregnancy until 38 weeks' gestation, with delivery of a healthy infant.

Coarctation of the aorta in infancy.

Twenty-five infants under 1 year of age (mean weight 3.4 kg) underwent repair of coarctation of the aorta between the years 1965 and 1982. Three patients had coarctation only, three had coarctation with patent ductus arteriosus (PDA), and 19 had associated intracardiac anomalies. Eleven patients underwent resection of the aorta and end-to-end anastomosis. Eight had subclavian flap arterioplasty, five had patch graft arterioplasty, and one had subclavian-to-aortic anastomosis. Additional procedures were performed on seven patients: banding of the pulmonary artery on one, repair of total anomalous pulmonary venous drainage on one, mitral valve replacement on one, aortic valvotomy on one, and aortic valvotomy plus pulmonary artery banding on one. Twenty-one survived the operation. All patients who died had associated intracardiac anomalies. The 21 survivors have been followed from 3 months to 13 years, with three late deaths that were associated with intracardiac anomalies. Five of the survivors underwent additional second operations: one had repair of the re-coarctation, one had replacement of the prosthetic mitral valve, one had aortic valvotomy, and two had pulmonary artery debanding and closure of a ventricular septal defect. Two of the 18 surviving patients have mild hypertension associated with a residual gradient. The others are in good health.

A prospective evaluation of pediatric patients with syncope.

Fifty-eight children with syncope were evaluated prospectively to determine the characteristics of syncope in the pediatric age group and the yield of various diagnostic tests. The age at first syncope ranged from 0.5 to 15 years. Twenty-five children presented after a single episode and 33 after multiple episodes. Ten had a history of breath-holding spells. Nineteen had a family history of syncope. A diagnosis was established in 53 patients (91%): vasodepressor (31), cardioinhibitory (13), tussive (3), hyperventilation (2), and mixed syncope (4). In five patients (9%), the cause remained unknown. The diagnosis was established from the history in 45 cases, by a positive oculocardiac reflex in 11, and by the head-up tilt test in four. We conclude that the cause of most cases of pediatric syncope is vasodepressor or cardioinhibitory and can be diagnosed by good history-taking. Costly evaluations are rarely necessary.

Transcatheter closure of secundum atrial septal defects with the amplatzer septal occluder: early experience.

BACKGROUND: Secundum atrial septal defect is a common congenital heart defect that causes right heart volume overload and produces symptoms usually after the third decade of life. Treatment until the last few years has been open heart surgery. OBJECTIVES: To review our early experience with transcatheter closure of ASD2 using the Amplatzer septal occluder. METHODS: Between November 1999 and February 2000, 20 children and young adults with a median age of 9.1 years (4.2-35.1 years) were referred for transcatheter closure of ASD2. Diagnosis was established by transthoracic echocardiography. Implantation was performed under general anesthesia through the femoral vein with the guidance of transesophageal echocardiography and fluoroscopy. Femoral arterial puncture was performed for blood pressure monitoring during the procedure. The device size chosen was similar to the balloon-stretched diameter of the ASD2. RESULTS: Implantation was completed successfully in 18 patients. Two patients were referred for elective surgery: one had an unsuitable anatomy for transcatheter closure by TEE in the catheterization laboratory and the device could not be implanted properly, the other patient had a large multiperforated septal aneurysm that was retrieved. Mean ASD2 diameter by TTE and TEE was similar (13.9 +/- 3 mm, 13.4 +/- 3.5 mm) and mean stretched diameter was 18.3 +/- 4.3 mm. Mean Qp:Qs (pulmonary flow:systemic flow) was 2.2 +/- 0.6. Mean fluoroscopy time for the procedure was 14.8 +/- 4.8 minutes. The patients were discharged the day after the procedure. Four patients had a tiny leak immediately post-procedure, and none had a leak at one month follow-up. The only complication was a small pseudoaneurysm of the femoral artery in one patient, that resolved spontaneously. CONCLUSIONS: Transcatheter closure of ASD2 with the Amplatzer septal occluder is a safe and effective alternative to surgical closure. Long-term outcome has to be evaluated.

Anomalous origin of the left coronary artery from the pulmonary artery: diagnosis and postoperative follow up.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital malformation that presents a diagnostic challenge to the pediatrician and pediatric cardiologist. Although surgical repair is always indicated, the optimal technique has yet to be determined. OBJECTIVES: To review our experience with the diagnosis of children with ALCAPA and to assess short to midterm surgical results. METHODS: Between 1992 and 1998, 13 infants and children (2 months to 15 years) were treated for ALCAPA at our medical center. Eight were diagnosed during the first year of life; all were symptomatic and had severe dysfunction of the left ventricle. The five patients diagnosed at an older age had normal myocardial function. Diagnosis was established by echocardiography alone in seven patients; six required catheterization (one infant and all older patients). Surgery was performed in 12 patients to establish dual coronary artery system: 7 underwent the Takeuchi procedure and 5 had re-implantation of the anomalous left coronary artery. RESULTS: One infant died shortly after diagnosis before surgical repair was attempted, and one died postoperatively. Four patients required additional surgery: three for late complications of the Takeuchi procedure and one valve replacement for mitral insufficiency. Recent evaluation revealed good global left ventricle function in all patients except for one, who is still within the recovery phase and shows gradual improvement. However, most patients who presented with severe myocardial dysfunction upon diagnosis still display abnormal features such as echo-dense papillary muscles or evidence of small akinetic segments. In this group, early repair was associated with faster myocardial recovery. CONCLUSIONS: The diagnosis of ALCAPA remains a clinical challenge to the pediatrician and cardiologist. Diagnosis can be established echocardiographically, and early diagnosis and treatment may lead to faster myocardial recovery. The preferred surgical method appears to be re-implantation of the ALCA. The chance for good recovery of global ventricular function is high even in the sickest patients, nonetheless abnormal myocardial features can be identified even years after surgery.

The Ross operation: initial Israeli experience.

BACKGROUND: The need for aortic valve replacement in children and young adults poses a special problem to cardiologists and surgeons. Replacing the sick aortic valve with the patient's pulmonary valve as described by Ross has proven to be a good option in this special age group. OBJECTIVE: To review our initial experience in order to assess the short-term results. METHODS: From January 1996 to June 1999, 40 patients (age 8 months to 41 years) underwent aortic valve replacement with pulmonary autograft. Indications for surgery were congenital aortic valve disease in 30 patients, bacterial endocarditis in 5, rheumatic fever in 3, and complex left ventricular outflow tract obstruction in 3. Trans-esophageal echocardiography was performed preoperatively and post-bypass in all patients, and transthoracic echocardiography was done prior to discharge and on follow-up. RESULTS: There was no preoperative or late mortality. All patients remain in functional class I (New York Heart Association) and are free of complications and medication. None showed progression of autograft insufficiency or LVOT obstruction. Homograft insufficiency in the pulmonary position has progressed from mild to moderate in one patient, and three developed mild homograft stenosis. CONCLUSIONS: The Ross procedure can be performed with good results in the young population and is considered an elegant surgical alternative to prosthetic values and homografts.

[Transcatheter closure of atrial septal defect].

Isolated secundum atrial septal defect is one of the most common congenital heart defects. Surgical closure is the treatment of choice but is associated with a chest scar, some morbidity and a relatively long recovery and the use of cardiopulmonary bypass. Transcatheter closure of secundum atrial septal defect is therefore an attractive approach. 3 children, aged 5-10 years, underwent successful transcatheter closure of moderate to large central atrial septal defects with the Cardioseal device. The procedures were performed under x-ray and transesophageal echocardiographic guidance. Our initial experience, and that of others, indicates that transcatheter occlusion of secundum atrial septal defects is safe and effective and can be an appropriate alternative in approximately 60% of patients.