Dolores A. Vitullo

Echocardiographic evidence for impaired myocardial performance in children with type I diabetes mellitus

Thirty-three children with type I diabetes mellitus and 51 normal children underwent M-mode echocardiography. Abnormalities of myocardial performance were present in many of the diabetic children. The mean end-systolic volume of the left ventricle was greater in diabetics compared to control subjects. Mean ejection fraction, minor axis shortening, and velocity of circumferential fiber shortening were decreased in the diabetics. There was no evidence of increased myocardial mass in these diabetic children. There was no correlation between myocardial dysfunction, clinical assessment of control, or glycohemoglobin in the diabetic children.

Alternatives in biventricular repair of double-outlet left ventricle

Wide variation in morphology of double-outlet left ventricle allows numerous surgical alternatives that require sorting out to develop a more organized approach. There is a high association between tricuspid abnormalities and right ventricular hypoplasia with double-outlet left ventricle that calls for either Fontan-type procedure or biventricular repair. With pulmonic stenosis, biventricular repair has been accomplished using right-sided conduits. When pulmonic stenosis is mild or absent, repair techniques without conduits depend on the commitment of the ventricular septal defect (VSD). With subaortic VSD and mild pulmonic valvar stenosis, we successfully performed translocation of the main pulmonary artery and valve to the right ventricle on 2 patients (ages 32 and 8 months). Both patients are doing well 2 years and 1 year postoperatively. Others have successfully connected the right ventricle to the pulmonary artery with intraventricular baffle by enlarging a subaortic VSD or when the VSD is either subpulmonic or doubly committed. With subaortic VSD, although it has not been reported, biventricular repair can also be accomplished using a right ventricle-to-aorta baffle combined by either atrial or arterial switch. We believe that a simplified management plan can be formed in double outlet left ventricle based on the size of the right ventricle, presence of pulmonic stenosis, and commitment of the VSD. Whenever possible, translocation of the main pulmonary artery and valve or intraventricular repair should be accomplished in double-outlet right ventricle minimizing the use of right-sided conduits and reoperation.

Neonatal Lupus with Congenital Atrioventricular Block and Myocarditis

This is a case of a child with neonatal lupus and congenital atrioventricuiar (AV) block, born to a mother with asymptomatic, systemic lupus erythematosus (SLE). The child, despite pacemaker insertion, died of septicemia and myocarditis at the age of three months. Although the association of neonatal lupus with congenital AV block is well‐recognized, there are only few pathologic studies of the conduction system reported in the literature. This is such a study in which we emphasize that, due to an altered immune system in the child, septicemia may be the cause of death in some cases.

FENESTRATED FONTAN PROCEDURE : EVOLUTION OF TECHNIQUE AND OCCURRENCE OF PARADOXICAL EMBOLISM

Abstract. The Fenestrated Fontan procedure (FFP) has improved outcome in high risk patients. The technique is evolving, however, and complications are not fully known. Over a 3-year period 13 patients (mean age 35 ± 29 months) underwent an FFP in our institution. In the first two patients the fenestration had to be created because of high right atrial pressure and low cardiac output; in 11 patients the FFP was planned. In three patients the sutures for the adjustable fenestration were crossing the defect. In 10 patients, purse-string sutures were placed around but not across the defect. Because large fenestrations were created in 11 patients (8–12 mm) Glenn shunts were performed to improve arterial saturation. The postoperative course was relatively uneventful, with chest tubes being removed 1–8 days (mean 4 ± 3 days) postoperatively and the hospital stay ranging from 7 to 27 days (mean 14 ± 6 days). One patient had bleeding and another had a mediastinal abscess. The first patient died (7.6%) because of hemodynamic instability due to prolonged cardiopulmonary bypass from the creation and enlargement of the fenestration. One patient had a paradoxical cerebral embolism from clots that formed on the sutures crossing the fenestration. Because of this problem the remaining patients were placed on salicylates while awaiting closure of their fenestration. All 12 patients had their fenestrations closed, performed under local anesthesia in 9, at mediastinal abscess drainage in 1, and spontaneously in 2. We conclude that creation of large fenestrations in combination with Glenn shunts and the use of adjustable fenestrations are viable modifications of the FFP. The use of purse-string sutures around the fenestration and antiplatelet drugs can probably minimize the occurrence of paradoxical embolism.

Regression of hypertrophic cardiomyopathy after modified Konno procedure

BACKGROUND Septal myotomy-myectomy has been known to decrease the incidence of sudden death and produce regression in hypertrophic obstructive cardiomyopathy. Use of beta-blockers or calcium-channel blockers generally does not cause regression of the disease. METHODS Having successfully performed modified Konno procedures in 13 patients with effective relief of diffuse subaortic stenosis, we applied the procedure in 2 patients with hypertrophic obstructive cardiomyopathy. Both patients (18 and 12 years old, respectively) presented with syncope, angina at rest, and dyspnea despite being on calcium channel blocker therapy. The echocardiographic outflow gradients were 66 mm Hg and 88 mm Hg, respectively, with moderate mitral regurgitation. RESULTS Both patients had uneventful postoperative course. At 2 years and 1.5 years postoperatively, both patients were free of angina and syncopal episodes. Echocardiography showed absence of outflow gradients and mitral regurgitation. In 1 patient the septal and posterior wall thickness decreased from 3.4 and 1.7 cm preoperatively to 2.6 and 0.9 cm, respectively, postoperatively. In the other patient, the thickness decreased from 2.4 and 0.9 cm preoperatively to 0.8 and 0.7 cm, respectively, postoperatively. Left atrial diameter decreased from 5.4 to 4.7 cm in 1 patient, 3.5 to 2.6 cm in the other. CONCLUSIONS We believe that the modified Konno procedure could produce more effective relief of obstruction and, therefore, significant regression and further reduction in sudden death in hypertrophic obstructive cardiomyopathy. On the basis of our experience, albeit limited, we encourage its application.

Cost-Effectiveness of Transaxillary Muscle-Sparing Same-Day Operative Closure of Patent Ductus Arteriosus

Transaxillary muscle-sparing patent ductus arteriosus closure performed as same-day surgery is described in 10 patients. This approach provides a superb cosmetic result while obviating the need for thoracostomy tube placement.

Early pulmonary homograft failure from dilatation due to distal pulmonary artery stenosis

Early progressive pulmonary homograft insufficiency developed in an 11-month-old infant after repair of truncus arteriosus because of dilatation secondary to the presence of residual distal pulmonary artery stenosis and hypoplasia. Before repair, the pulmonary artery branches were discontinuous, with the right pulmonary artery being somewhat hypoplastic and originating from the trunk, and the left pulmonary artery supplied by a modified Blalock-Taussig shunt created in the newborn period. At repair, a pulmonary homograft was used to connect the branches. Progressive cardiomegaly and oxygen dependance occurred 3 weeks postoperatively. Cardiac catheterization showed systemic right ventricular pressure, severe homograft insufficiency, and residual distal pulmonary artery stenosis and hypoplasia. On reoperation at 3 months postoperatively, the homograft annulus diameter increased from 14 mm to 16 mm. Dilatation and insufficiency probably occurred because the right ventricle and homograft distal to the obstruction functioned as a unit during systole. The problem might have been minimized with the use of aortic homograft, which is thicker, or annular reinforcement with a synthetic material.

1121 ECHOCARDIOGRAPHIC EVIDENCE FOR IMPAIRED MYOCARDIAL PERFORMANCE IN CHILDREN WITH TYPE I DIABETES MELLITUS

Myocardial performance was assessed by M-mode echocardiography in 33 children (6 8/12-19 3/12 yr) with Type I diabetes mellitus and in 51 normal children (6 2/12-18 8/12). Left ventricular end systolic dimension (LVESD), and left ventricular end systolic volume/M2 (LVESV/M2) were greater in diabetics than controls. Left ventricular ejection fraction (LVEF), minor axis shortening (MAS), and velocity of circumferential fiber shortening (VCF) were less in diabetics than controls.Hgb A1 levels in children with diabetes (15.5±0.6%, range 10.1-22.2%, normal 5.9-8.7%) correlated with clinical assessment of control (p<.01). Age, Hgb A1, duration of diabetes, and clinical assessment did not predict myocardial function. We conclude that there is impaired myocardial contractility in some children with insulin-dependent diabetes not correlated with duration of diabetes, age, clinical assessment of control, or Hgb A1. The long-term significance of this finding and the effect of improved control remain to be assessed.

1058 INTRARENAL BLOOD FLOW (RBF) AFTER ENDOtOXIC SHOCK (ES)

The purpose of this study was to determine distribution of IRBF in infant puppies in response to ES. Studies were performed in 18 mongrel puppies age 10-20 days. Cardiac output (CO), total renal blood flow (RBF) and IRBF was determined by dye dilution and the injection of 25 μ radiolabeled microspheres into the left ventricle. ES was produced by IV injection of 10 mgm/kg of E coli 0111 B4.These data demonstrate that RBF falls significantly 5 min. after ES and begins to increase by 20 min. OC and IC flow is initially unchanged; in late ES, IC flow is maintained as a result of a significant fall in OC flow. This response of IRBF may play a role in renal functional response to ES.