Eugenie F. Doyle

Natural history of rheumatic aortic regurgitation. Criteria predictive of death, congestive heart failure, and angina in young patients.

The medical courses of 174 young patients with aortic regurgitation were followed prospectively for a median of 10 years. The data were analyzed by life-table methods; congestive failure and angina, as well as death, were considered as end points, since the occurrence of the former is considered sufficient indication for aortic valve replacement. Thirty-one patients developed the triad of moderate or marked left ventricular enlargement, two or three electrocardiographic abnormalities, and abnormal blood pressure. Thirty-three percent of these patients either died or had failure or angina within 1 year, 48% within 2 years, 65% within 3 years, and 87% within 6 years from the acquisition of the triad. The 71 patients with none of the above features had uneventful courses. Of the 71 patients with one or two features only seven either died (three) or became symptomatic. These data are useful for patient selection for surgery before symptoms appear.

Absence of a Primary Division of the Pulmonary Trunk An Ontogenetic Theory

An ontogenetic theory is proposed to explain the absence of a primary division of the pulmonary trunk on the basis of an error in the septation of the truncus arteriosus, and the two common varieties of the syndrome are discussed in detail. It is suggested that:Both lateral pulmonary arteries are present in this syndrome, but one of them arises anomalously from the systemic circulation.The aberrant origin of one lateral pulmonary artery derives from the dorsal shifting of one of the two ridges responsible for the septation of the truncus arteriosus. The sixth arch on the side of the dorsorotated truncal ridge then becomes incorporated into the newly formed ascending aorta.The combination of tetralogy of Fallot, right aortic arch, and absent left primary pulmonary division results from a single primordial error: a dorsorotation of the left ridge extending throughout the truncoconus.The initial portion of the aberrant (systemic) pulmonary artery may obliterate postnatally, thus impairing maturation of the terminal portion. This would explain the frequent failure to demonstrate the artery by angiography and, occasionally, at dissection.

Syndrome of anomalous venous drainage of the right lung to the inferior vena cava

Abstract Anomalous venous drainage of the right lung to the inferior vena cava is associated with abnormal lobation and bronchial division, hypoplasia of the lung and the pulmonary artery, and displacement of the heart to the right. There may also be branches of systemic arteries entering the lung, cystic bronchial diverticula, diaphragmatic defects, or congenital heart disease. A review of the 67 cases previously reported shows that the syndrome is highly variable and that one may be misled by reports of small series of patients. Three new cases in children have been described. All had an unusual degree of hypoplasia of the right lung, which probably accounts for absence of the typical “scimitar sign” on their chest roentgenograms. Angiocardiograms obtained routinely in patients with hypoplasia of a lung may be expected to reveal more cases of vascular anomalies of this kind. Bronchospirometry is the best method for evaluating the degree of functional impairment. Young people have few symptoms even when the pathologic changes are severe. Anastomosis of the anomalous vein to the left atrium is indicated if physiologic studies show that it carries a significant flow of blood, and if lung resection is not indicated because of local symptoms.

Endocarditis Complicating Open-Heart Surgery

BACTERIAL ENDOCARDITIS following intracardiac surgery for congenital defects with use of extracorporeal circulation is a new entity, as old only as open intracardiac surgery. The reported experience is therefore meager. Suggestions regarding its pathogenesis, recognition, behavior, management, and prevention are few. Heins and Lindel reported five cases of bacterial endocarditis in a series of 205 heart defects repaired with extracorporeal circulation. They stress the facts that the classical signs usually associated with bacterial endocarditis are absent and that the bacterial flora encountered is unusual and frequently antibiotic resistant. Two of their patients acquired their infection with achromobacter from a contaminated heart-lung machine when ethylene oxide sterilization rather than autoclaving was employed. One of their five cases died. Of additional interest, fever occurring shortly after surgery was the only clue that ultimately led to diagnosis. Mandel et al.2 first reported a case of bacterial endocarditis following repair of an interventricular septal defect. The operation was performed under hypothermia and coronary perfusion. The infecting organism was Staphylococcus aureus, and the patient died. Their review of the literature revealed 30 cases of endocarditis following operations for repair of congenital and acquired cardiac lesions but none of these was done with extracorporeal circulation. Teitel and Florman3 reported an unusual case of Pseudomonas aeruginosa infection on a silk suture used in the repair of an atrial septal defect with extracorporeal circulation. The infection resisted medical therapy until

Observations on rheumatic nodules over a 30-year period

Summary 1. During a 30-year period from 1928 to 1958, 948 episodes of rheumatic carditis were observed on the Childrens Cardiac Service, Bellevue Hospital. 2. In the first half of this 30-year period from 1928 to 1942 (Period A), 98 deaths occurred in the course of 599 attacks of carditis for a mortality rate of 16.4 per cent. During the second half from 1943 to 1958 (Period B), 15 deaths occurred during 349 attacks of carditis for a mortality rate of 4.3 per cent. 3. The percentage of attacks of carditis with nodules during the two periods showed no significant difference. During Period A nodules were present in 68 of the 599 episodes of carditis (11.3 per cent) and in Period B in 33 of the 349 episodes of carditis (9.4 per cent). 4. Nodules occurred only in patients with active carditis. Ninety-six patients exhibited nodules on 101 occasions. In 5 patients nodules were observed twice in separate attacks. 5. The severity of carditis was not correlated directly with the number of nodules. The mortality rate among patients with a few nodules, 10 or less, was greater than that among patients with 11 to 30 nodules. 6. Nodules rarely were observed during the first weeks of an acute rheumatic attack. They usually appeared after a month of illness and occurred most frequently in patients in whom rheumatic activity persisted for many weeks or months.

Use of saphenous vein allografts for aortopulmonary artery anastomoses in neonates with complex cyanotic congenital heart disease

SummaryA saphenous vein allograft was used to create an aortopulmonary communication in 16 infants with cyanotic congenital heart disease and ductus-dependent pulmonary blood flow. These grafts measured from 3 to 8 mm in diameter and were placed between the aorta and main pulmonary artery in eight patients, between aorta and right pulmonary artery in eight, and between aorta and left pulmonary artery in one (one child had two grafts). Before heparin was used, early in the series, four of these grafts occluded and three of the four infants died during attempted revision. Another infant died early from renal failure. Late mortality has claimed four: one from cerebral hemorrhage, two from hypoxia, and one at open-heart surgery for repair. There are eight late survivors (50%).Most of the allografts were used before small diameter Gore-Tex was available; in more recent patients, 4- to 6-mm Gore-Tex grafts have been used. In our most recent patient, however, the attempt to place a Gore-Tex graft was unsuccessful, but the more pliable saphenous vein graft was readily placed and an adequate shunt obtained. Both the saphenous vein graft and the Gore-Tex have the advantage of providing pulmonary flow without the higher risk of congestive failure or pulmonary hypertension seen in patients with a Waterston or Potts anastomosis. They are easier to perform, require less anesthesia time than the Blalock-Taussig shunt, last as long as the Blalock-Taussig when done under similar conditions, and are easy to take down at the time of total repair.

Repair of posterior left ventricular aneurysm in a six-year-old boy.

Left ventricular aneurysms and diverticula are rarely encountered in the pediatric age group. This paper reports a case of congestive heart failure and mitral regurgitation in a 6-year-old boy with a large posterolateral left ventricular aneurysm. Complete repair was successfully performed by excision of the aneurysm and Dacron patch reconstruction of the left ventricular free wall. The patch extended onto the posterior annulus of the mitral valve, thus restoring the mitral valve to normal geometry and correcting the mitral insufficiency. The surgical literature on congenital cardiac diverticula and acquired aneurysms in children is reviewed and summarized.

Masked abnormal drainage of the inferior vena cava into the left atrium.

A 6 year old boy with a large atrial septal defect, partial anomalous pulmonary venous drainage and unrecognized anomalous insertion of the inferior vena cava into the left atrium had cyanosis after closure of the atrial defect. Repeat study revealed direct drainage of the inferior vena cava into the left atrium with moderate arterial oxygen desaturation. At repeat operation an unusual positioning of the inferior vena cava was seen. After reopening of the atrial defect, the pulmonary venous and systemic venous drainage anomalies were identified. A Dacron patch was inserted so as to divert flow to the proper atrium. Repeat catheterization 3 months after operation revealed a normal heart with no obstruction; arterial oxygen saturation was normal. The child has continued to do well 3 years after operation.

Repair in infancy of tetralogy of Fallot with absence of the leaflets of the pulmonary valve (absent pulmonary valve syndrome) using a valved pulmonary artery homograft

Two infants, aged eight days and four months, with tetralogy of Fallot and absence of the leaflets of the pulmonary valve, presented with acute respiratory distress and congestive heart failure. Primary repair was performed using a cryopreserved pulmonary arterial homograft. Both infants survived and are doing well three and two years later. A review of the surgical procedures attempted in this symptomatic group of patients is presented together with a discussion of the pathophysiology of this defect.

Results of urgent or emergency repair for symptomatic infants under one year of age with single or multiple ventricular septal defect

Repair of ventricular septal defects (VSDs) in symptomatic infants aged < 1 year previously had a high mortality and led to the use of pulmonary artery banding for many years. 1 As improvements in techniques (surgical, open-heart, instrumentation, myocardial preservation, anesthesia and postoperative care) occurred, the use of pulmonary artery banding for an isolated VSD decreased, and in many centers stopped completely.2 We report the results of VSD repair in 54 unselected infants who presented with congestive heart failure that had limited or no improvement with medical treatment. The types of VSDs and the associated defects are reported, as well as in-hospital and follow-up mortality and morbidity.