Alexander J. Muster

Coronary Artery Fistulas in Infants and Children: A Surgical Review and Discussion of Coil Embolization

BACKGROUND Coronary artery fistula (CAF) is a rare congenital anomaly that can be complicated by intracardiac shunts, endocarditis, myocardial infarction, or coronary aneurysms. Recent reports have emphasized the efficacy of percutaneous transcatheter techniques. The purpose of this article is to review a 28-year surgical experience with CAF as a standard for comparison and to discuss the emergence and efficacy of transcutaneous catheter coil embolization as an alternative form of therapy. METHODS From 1968 to 1996, 17 patients (age, 6 weeks to 16.5 years; mean age, 5.5 years) were diagnosed with CAF: 8 of 12 by echocardiography and 17 of 17 by cardiac catheterization. All patients with isolated CAF (n = 13) were asymptomatic despite significant clinical, electrocardiographic, and chest roentgenographic findings in 10. Sixteen had congenital CAF and 1 had acquired CAF after tetralogy of Fallot repair with injury of the anomalous left anterior descending coronary artery. Associated anomalies included tetralogy of Fallot (2), atrial septal defect (1), and patent ductus arteriosus (1). Nine fistulas originated from the right coronary artery and eight from the left. Drainage was to the right ventricle (9), right atrium (4), pulmonary artery (3), and left atrium (1). RESULTS All patients had a median sternotomy with epicardial or endocardial ligation. Cardiopulmonary bypass was used in 8; 1 of these (iatrogenic CAF) required distal internal mammary artery bypass graft. There were no operative or late deaths. Follow-up evaluation by physical examination (17), echocardiography (8), and catheterization (2) showed no evidence of recurrent or residual CAF. A retrospective review of the 16 available cine cardioangiograms showed that coil embolization was possible in, at most, 6 patients. CONCLUSIONS Early surgical management of CAF is a safe and effective treatment resulting in 100% survival and 100% closure rate. Transcatheter embolization is a reasonable alternative to standard surgical closure in only a very small, select group of patients. These surgical results should be considered the standard against which transcatheter techniques are compared.

Discrete subvalvular aortic stenosis in childhood: Study of 51 patients

Fifty-one children with discrete subvalvular aortic stenosis were studied between 1951 and 1974. The three anatomic types of obstruction found were the thin membranous type (43 cases), the fibromuscular collar type (5 cases) and the tunnel type (3 cases). The obstruction was usually severe, and the median left ventricular to aortic systolic pressure gradient was 90 mm Hg. Progressive obstruction with an increasing gradient was documented in 10 patients by serial cardiac catherizations. Significant associated cardiac defects, present in 57 percent of patients, often masked the typical clinical and cardiac catheterization features of subaortic stenosis. The stenosis was often not discovered until after surgery for the associated defect. Forty patients underwent surgical resection of the discrete subaortic obstruction. After surgery significant left ventricular to aortic pressure gradients can be found at postoperative cardiac catheterization. These gradients may reflect inadequate resection of the more complex discrete obstructions or represent proliferation and regrowth of the previously resected subvalvular fibrous tissue. The criteria for operability of discrete subaortic stenosis should be the angiographic demonstration of a discrete subvalvular diaphragm and the presence of a resting left ventricular to aortic systolic pressure gradient of 40 mm Hg or more.

Pulmonary vascular disease in complete transposition of the great arteries: A study of 200 patients☆

Abstract Lung specimens of 200 patients with transposition of the great arteries were examined microscopically for evidence of pulmonary vascular disease. In patients with an intact ventricular septum or a small ventricular septal defect, advanced pulmonary vascular disease was uncommon; only 9 of 107 such patients (8.4 percent) demonstrated greater than grade 2 (Heath-Edwards) pulmonary vascular disease. A persistent large patent ductus arteriosus appeared to promote progressive pulmonary vascular disease in this group since each of the five infants less than 1 year of age with grade 3 or 4 disease had this lesion. In contrast, pulmonary vascular disease was common in patients with a large ventricular septal defect; 37 of 93 patients (40 percent) with this defect had greater than grade 2 pulmonary vascular disease. Among patients more than 1 year of age, 26 of 35 (75 percent) had grade 4 disease. The catheterization data suggest that the calculated pulmonary vascular resistance may underestimate the degree of disease, probably by overestimating the pulmonary blood flow (Fick method). Pulmonic stenosis appeared to protect the lungs from progressive pulmonary vascular disease, and pulmonary arterial banding was protective when performed before age 6 months. Our studies indicate that a persistent large patent ductus arteriosus should be closed as early as possible in view of its association with advanced pulmonary vascular disease in these patients. In infants with a large ventricular septal defect, pulmonary arterial banding or corrective surgery with closure of the defect should be performed between the ages of 4 and 6 months to prevent progressive pulmonary vascular damage.

Balloon angioplasty-branch pulmonary artery stenosis: Results from the Valvuloplasty and Angioplasty of Congenital Anomalies Registry

Balloon angioplasty for branch pulmonary artery stenosis was reported from 27 institutions to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. One hundred eighty-two procedures were performed in 156 patients ranging in age from 0.2 to 46.2 years (mean 7.7). Short-term angiographic appearance, hemodynamic results and immediate complications were recorded. Vessel dimension at the site of stenosis increased from 4.5 +/- 2.0 (mean +/- standard deviation) to 6.8 +/- 3.0 mm (p less than 0.001) with greater increases in vessel dimension at the site of stenosis if the balloon diameter was greater than 3 X the original dimension of the stenosis. There was no significant benefit related to age or prior surgical intervention. The mean peak systolic pressure gradient was reduced from 49 +/- 25 to 37 +/- 26 mm Hg (p less than 0.001) and pressure proximal to the stenosis decreased from 69 +/- 25 to 63 +/- 24 mm Hg (p less than 0.001). Complications occurred in 21 patients and included vessel rupture and death in 2 patients, vessel perforation or rupture with survival in 3, cardiac arrest and death in 1, paradoxical embolism and death in 1 and low output and death in 1. Balloon angioplasty for branch pulmonary artery stenosis increases vessel dimension at the site of stenosis, reduces systolic pressure gradient and to a minor degree, reduces proximal pressure. Long-term outcome and potential complications are as yet uncertain.

Relation between preoperative left ventricular muscle mass and outcome of the fontan procedure in patients with tricuspid atresia

The relation between preoperative left ventricular muscle mass and clinical outcome of the Fontan procedure was evaluated retrospectively in 22 patients with tricuspid atresia who were selected for this physiologic surgical correction by conventional hemodynamic criteria. Patients were divided into two groups: group A (excellent or good outcome) and group B (poor outcome or death) based on the clinical course assessed up to 9.5 years postoperatively. Thirteen of 22 group A patients did not have prolonged, clinically significant, systemic venous hypertension and were not on long-term diuretic drug therapy. Nine of 22 group B patients either had clinically significant systemic venous hypertension, required long-term diuretic drug therapy or died (3 patients). Age at surgery, pulmonary arteriolar resistance, left ventricular ejection fraction, end-diastolic volume, end-diastolic pressure, systemic oxygen saturation and pulmonary to systemic blood flow ratio (Qp/Qs) were not statistically different between the two groups. Left ventricular muscle mass, both in group A patients (92 +/- 31 g/m2) and in group B patients (146 +/- 61 g/m2), was greater than the normal mean value (p less than 0.01 and p less than 0.001, respectively). Left ventricular muscle mass in group B was significantly greater than in group A (p less than 0.01). Furthermore, left ventricular muscle mass/end-diastolic volume (mass/volume) ratio, reflecting the extent of left ventricular hypertrophy relative to volume overload, was significantly greater in group B (1.1 +/- 0.28) than in group A (0.84 +/- 0.21) (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Neonatal polycythemia with transient cyanosisand cardiorespiratory abnormalities

Ten infants with transient plethora and cyanosis in the newborn period are presented,with findings suggestive of transient cardiovascular strain. A search for common etiologic factors revealed considerable elevation in the hematocrit and hemoglobin levels consistent with neonatal polycythemia. The several sources of neonatal hypervolemia and polycythemia are discussed; it seems likely that an excessive transfusion of placental blood at delivery is an important factor. Spontaneous improvement generally occurs although phlebotomy has been therapeutically applied in two instances.

Restrictive ventricular septal defect: How small is too small to close?

Historically, indications for ventricular septal defect closure have included congestive heart failure, pulmonary hypertension, aortic insufficiency with or without aortic valve prolapse, and prior bacterial endocarditis. However, controversy exists as to how the lifetime risk of an isolated, nonoperated restrictive ventricular septal defect compares with the risk of surgical closure in an asymptomatic child. Between 1980 and 1991, cardiac catheterization and elective ventricular septal defect closure (age > 1 year, pulmonary to systemic flow ratio < 2.0) were performed in 141 patients aged 1 to 23 years (mean age, 6.1 +/- 4.7 years). Mean systolic pulmonary artery pressure was 26.9 +/- 13.0 mm Hg, and mean pulmonary to systemic flow ratio was 1.6 +/- 0.3. Aortic valve prolapse was present in 63 patients (45%), aortic insufficiency was present in 25 (18%), and 5 (3.5%) had prior bacterial endocarditis. There were no early or late deaths or major morbidity. No patient required a ventriculotomy to accomplish ventricular septal defect closure. Mean postoperative intensive care unit stay was 1.3 +/- 0.9 days, and mean hospital stay was 5.5 +/- 1.9 days. There were no instances of permanent complete atrioventricular dissociation, reoperations for bleeding, postoperative wound infections, or reoperations for residual or recurrent ventricular septal defect. These improved results justify a reevaluation of historic indications for ventricular septal defect closure.(ABSTRACT TRUNCATED AT 250 WORDS)

Hypoplastic left ventricle syndrome: Analysis of thirty autopsy cases in infants with surgical considerations☆

Abstract Physiologic and anatomic data of 30 infants with hypoplastic left ventricular syndrome who underwent autopsy have been reviewed, and a possibly effective surgical palliative measure is proposed. Provision of an unobstructed interatrial communication for free left to right shunting of pulmonary venous blood to the right side of the heart is the first objective. Provision of continuing right to left shunt at the ductus level, sustained by high resistance to pulmonary artery blood flow, is the second. Surgical banding of both pulmonary arteries distal to the ductus is recommended.

Coarctation of the Aorta in Infancy

Material from 78 infants with coarctation and congestive heart failure during the first 6 months of life was reviewed. Three fourths of these infants had combined aortic lesions, that is, coarctation, a narrow constrictive zone of stenosis, and tubular hypoplasia of the transverse arch, a proximal, long, uniformly narrow segment.It was the purpose of this study (1) to distinguish coarctation of the aorta from tubular hypoplasia by using pathologic and angiographic measurements, (2) to assess the relationship of these two entities and additional shunting cardiac defects with the occurrence of congestive heart failure in infancy, and (3) to evaluate the optimal therapeutic approach for symptomatic infants with coarctation.It was found that (1) coarctation of the aorta as an isolated anomaly is a relatively uncommon cause of congestive heart failure in infancy. In contrast, coarctation associated with tubular hypoplasia of the transverse arch or with additional cardiac defects is frequently associated with severe congestive heart failure. It was found also (2) that surgical management is far more successful than medical management alone in symptomatic infants with coarctation of the aorta and associated significant cardiac defects.

Expanding indications for pediatric coronary artery bypass

Pediatric coronary artery bypass has been done mostly for ischemic complications of Kawasaki disease. We reviewed our clinical experience between 1987 and 1994 with internal thoracic artery-coronary artery bypass in one infant and five children for varying indications. Indications for coronary bypass included Kawasaki disease (2), congenital left main coronary ostial stenosis, iatrogenic coronary cameral fistula, anomalous origin of the left coronary artery from the pulmonary artery, and single coronary artery traversing between the great arteries in a patient after cardiac transplantation. An additional cohort of 34 control patients of various ages and weights (1 day to 16.1 years, 2.6 kg to 62 kg) had angiographic measurements of the right coronary, left coronary, and left internal thoracic arteries with respect to the feasibility of performing coronary artery bypass. All six patients survived internal thoracic artery-left anterior descending coronary artery bypass without evidence of perioperative myocardial infarction. Postoperative angiographic studies in five and color Doppler echocardiography in one showed graft patency. Retrospective angiographic measurements in the 34 control patients showed that internal thoracic and coronary arteries are proportionately quite large in neonates and infants compared with those in older children and adolescents. Internal thoracic artery-coronary artery bypass should be considered for the expanding indications presented herein and when emergency intraoperative life-threatening situations present themselves. Long-term patency and reoperation rates have yet to be determined.