Roger B. Cole

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.

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.

Long-term results of aortopulmonary anastomosis for tetralogy of fallot: morbidity and mortality, 1946-1969.

A review of 340 patients with tetralogy of Fallot undergoing aortopulmonary anastomosis of the Potts-Smith type (descending aorta to pulmonary artery) has been made for assessment of the complications and long-term results (1946-1969) of this procedure. There were 30 operative deaths (9%) and 52 late medical deaths (20%) The symptomatic improvement has remained impressive in the survivors even up to 20 years after shunt surgery, with 85% of the patients having a functional classification of I or II.The major late complications have been related to anastomotic channels which were made or eventually became too large, resulting in congestive heart failure (20 late medical deaths) or in pulmonary hypertension with pulmonary vascular obstructive disease. These complications are not unique to the Potts operation, and may be anticipated in patients with excessively large ascending aorta to right pulmonary artery anastomoses. Careful patient follow-up is indicated and, in the event of congestive heart failure or evidence for pulmonary hypertension, an aggressive approach with either shunt take down and intracardiac repair or shunt revision should be considered.

Double-Outlet Left Ventricle with an Intact Ventricular Septum: Clinical and Autopsy Diagnosis and Developmental Implications

Clinical, laboratory, and anatomic findings were presented concerning the first clinically diagnosed and autopsy proved case of origin of both great arteries from the morphologically left ventricle.The salient pathologic findings were: normally interrelated chambers (noninverted) in situs solitus; no ventricular septal defect; infundibular atresia; infundibulum related only to the right ventricle; absence of infundibular musculature beneath the great arteries, with aortic-mitral and pulmonary-mitral fibrous continuity; semilunar valves side by side, aortic to the right and pulmonary to the left, and at approximately the same height; thick-walled and small-chambered right ventricle with endocardial fibroelastosis of the apical half of the ventricle; hypertrophy and enlargement of both atria and of the left ventricle; absence of the left coronary ostium; and a fistula between the right ventricular apex and the anterior descending coronary artery.The main developmental implications of this rare case appear to be as follows: (1) strong support is given for the differential conal growth hypothesis concerning the morphogenesis of great arterial interrelationships; (2) truncus arteriosus communis would appear to have atresia, not absence, of the subpulmonary infundibulum; (3) the truncal septum does extend a short distance below the semilunar valves; and (4) the conus arteriosus appears to be a separate cardiac segment, not part of the right ventricle and not part of the great artelies.

Surgical Management of Infants with Complex Cardiac Anomalies Associated with Reduced Pulmonary Blood Flow and Total Anomalous Pulmonary Venous Drainage

Eight infants with complex cardiac anomalies and pulmonary stenosis or atresia were noted to have obstructed total anomalous pulmonary venous drainage (TAPVD) either at the initial cardiac catheterization (Group 1; n = 2) or after creation of systemic-pulmonary artery shunts (Group 2; n = 6). The 2 patients in Group 1 underwent early repair of TAPVD (1 at 7 days, the other at 1 1/2 months of age) before any subsequent operation and are now doing well at 18 months of age. The 6 patients in Group 2 underwent repeat cardiac catheterization because of persistent severe cyanosis with faint or absent continuous murmur and were found to have patent shunts and obstructed TAPVD (1 mild, 5 severe). One patient who underwent repair of TAPVD at 2 1/2 months of age survived and is well at 2 years of age, whereas 4 patients who underwent repair at an average age of 6 months (age range, 3-16 months) subsequently died. The sixth patient, who did not undergo repair, remained severely cyanotic with hypoplastic pulmonary arteries in spite of repeated shunts. We feel that increased awareness of the possible association of TAPVD and reduced pulmonary blood flow in infants with complex cardiac defects, in combination with echocardiography, oxygen saturation studies, and angiography with prostaglandin E1 challenge, should lead to early diagnosis, avoidance of unnecessary systemic-pulmonary artery shunts, and increased survival rates in these infants.

Plasma cell granuloma of the heart.

A case of plasma cell granuloma arising in the free ventricular wall and involving the coronary arteries of a 10‐month‐old female infant is presented. Morphologic characteristics were comparable with those of plasma cell granulomas arising elsewhere. The histologic, immunohistochemical, and ultrastructural observations, and in particular the quiescent clinical course of the disease and its control with anti‐inflammatory medication, favor a nonneoplastic nature of the lesion. This report documents the presence of an intracardiac plasma cell granuloma for the second time in the medical literature.

Pulmonary vascular disease after systemic-pulmonary arterial shunt operations.

More than 1,100 patients underwent a systemic-pulmonary arterial shunt operation at the Childrens Memorial Hospital, Chicago, from 1946 to 1974. Of these patients, 294 were studied to assess the presence of pulmonary vascular disease and the time of its occurrence after surgery. They were selected either because lung tissue was available for histologic grading of pulmonary vascular disease (114 patients) or because hemodynamic studies were performed after the shunt operation (180 patients). The shunts, all patent at the time of the study, ranged in duration from 1 month to 23 years. After a subclavan-pulmonary arterial shunt (75 patients) there was little evidence of advatients) pulmonary vascular disease in either histologic C2 of 25 patients) or hemodynamic (1 of 50 patients) studies. After an aortic-pulmonary arterial shunt (44 Waterston, 175 Potts) there was small likelikhood of advanced pulmonary vascular disease with a shunt duration of less than 5 years (1 of 35 histologic, 0 of 27 hemodynamic studies). With a shunt duration of more than 5 years the incidence of advanced pulmonary vascular disease increased significantly (15 of 54 histologic, 17 of 103 hemodynamic studies). A mean pulmonary arterial pressure of 50 mm Hg or greater strongly suggests the presence of advanced pulmonary vascular disease. When a systemic-pulmonary arterial shunt has provided effective palliation with decreased pulmonary pressure there is small likelihood that advanced pulmonary vascular disease will develop, even with a long shunt duration of 20 to 25 years.

Pulmonary atresia with intact ventricular septum

Abstract Thirty-nine autopsy cases of Type i pulmonary atresia with an intact ventricular septum have been reviewed. Clinical correlation was available in 19 cases; catheterization and angiocardiographic data were included in 7. This complex is associated with an extremely thickwalled but minute capacity right ventricular chamber which is generally not amenable to correction by pulmonary valvotomy. Twelve patients underwent attempts at surgical palliation, including valvotomy and various systemicpulmonary artery anastomotic procedures. Only 3 patients survived the immediate postoperative period. All 3 had undergone aortopulmonary anastomosis, surviving, respectively, two months, fourteen months and three years postoperatively. Since hypoplasia of the right ventricular cavity is a major factor in limiting the surgical approach in this lesion, particular attention was given to the correlation of the electrocardiogram with right ventricular anatomy. Of 13 patients for whom 12-lead scalar electrocardiograms were available, 9 had left ventricular hypertrophy or left ventricular preponderance (absence of the expected right ventricular preponderance for age). The remaining 4 infants, all with equally small right ventricular chambers, had patterns of right ventricular or combined ventricular hypertrophy with varying degrees of right bundle branch block. In addition, an age-dependent progression in right ventricular potentials and occurrence of right bundle branch block were suggested from a review of individual and serial electrocardiograms. Nine infants with electrocardiographic left ventricular hypertrophy or preponderance were all under 3 months of age. In contrast, 3 of 4 infants with records of right ventricular or combined ventricular hypertrophy were 3 months or older. Thus, when a right ventricular or combined ventricular pattern was noted in Type i atresia, the electrocardiogram could not be relied upon to accurately mirror right ventricular chamber size. The results of operation for this malformation, regardless of the procedure attempted, are discouraging, particularly when contrasted with those for tetralogy with pulmonary atresia and tricuspid atresia at comparable ages. The factors that contribute to the high operative mortality are not adequately understood.

Ebstein's malformation of the tricuspid valve in the neonate. Functional and anatomic pulmonary outflow tract obstruction.

Abstract The clinical and physiologic data obtained in 4 infants with Ebsteins malformation and 2 with pulmonary atresia with intact ventricular septum and large volume right ventricle have been presented. These two conditions may be extremely difficult to differentiate in the neonatal period on clinical grounds alone, and catheterization and angiocardiographic studies are required for an accurate diagnosis. Functional pulmonary valve obstruction in neonates with Ebsteins malformation resulting from ineffective forward ejection of right ventricular blood and with little or no blood traversing a normal pulmonary valve has been demonstrated angiocardiographically. Anatomic obstruction of the right ventricular outflow tract by the large curtain-like, malaligned tricuspid valve leaflet has also been demonstrated. An accurate assessment of the anatomy and physiology of the pulmonary outflow tract is essential to therapeutic management.