Henry M Sondheimer

Mechanical limitation of pulmonary blood flow facilitates heart transplantation in older infants with hypoplastic left heart syndrome

OBJECTIVES Progression of pulmonary vascular disease limits heart transplantation for hypoplastic left heart syndrome (HLHS) to early infancy. Our objective was to assess the impact of bilateral pulmonary artery banding (PAB) on the operative courses of HLHS infants transplanted at ages older than 4 months. METHODS Courses of all HLHS patients in our center who remained listed to age >or=120 days before heart transplantation were assessed. Patients undergoing transplantation after standard management (control group) were compared to patients having a prior pulmonary blood flow limiting procedure (PAB group). RESULTS Of 16 identified patients, one crossed over to stage I Norwood on day 185 and died post-operatively. Fifteen patients were transplanted at age >or=120 days (control group n=9, PAB group n=6). Four PAB patients had open PA band placement. Two PAB patients underwent experimental percutaneous bilateral internal pulmonary artery flow limiting device insertion. The PAB group mean age at banding was 141+/-54 days, and mean interval from PAB to transplant was 35+/-31 days (range 1.5-68 days). No differences in age at transplant, weight at transplant, warm graft ischemia time or total graft ischemia time were detected between groups. Mean times of mechanical ventilation (control 143+/-69h vs. PAB 44+/-13h), inhaled nitric oxide (control 126+/-70h vs. PAB 37+/-9h), inotropic support (control 171+/-64h vs. PAB 87+/-17h), intensive care unit (ICU) stay (control 8.3+/-2.7 days vs. PAB 4.5+/-1.4 days), and hospital stay (control 10.4+/-3.9 days vs. PAB 7.0+/-1.1 days) were all lower in the PAB group (P<0.05 all comparisons). Two control patients died, three required extracorporeal membrane oxygenation (ECMO), and six did not tolerate primary chest closure. No PAB patient died or required ECMO. All PAB patients tolerated primary chest closure. All PAB patients had widely patent branch pulmonary arteries with no re-interventions to date. All hospital survivors remain alive (mean follow-up, control 50.2 months, PAB 11.5 months). CONCLUSIONS Pre-transplant mechanical limitation of pulmonary blood flow simplified management and reduced morbidity for HLHS patients undergoing heart transplantation at ages >or=4 months. This strategy extends the permissible transplant waiting time in older infants with HLHS.

Profound hypothermia with alpha-stat pH management during open-heart surgery is associated with choreoathetosis

A retrospective chart review was conducted to examine risk factors associated with the occurrence of choreoathetosis, a rare but significant complication of open-heart surgery in children. Ten children were identified as having developed choreoathetosis after cardiac surgery. Their charts were reviewed and compared with 33 age- and diagnosis-matched controls who underwent open-heart surgery during the same time period. Children with choreoathetosis reached lower rectal and esophageal temperatures (p = 0.0018 for both) and spent a greater portion of total bypass time at lower rectal and esophageal temperatures (p < 0.001 for both). Duration of cooling below 20°C esophageal temperature and PaCO2 at the end of the cooling period were significant predictors of choreoathetosis (p = 0.023 and p = 0.0497, respectively) in a logistic regression model, and a greater fraction of choreoathetosis patients had prior developmental delays (p = 0.017). No difference was found in the age at surgery, duration of bypass, aortic cross-clamp time, arterial pH, PaCO2 or mean arterial pressure. The combination of extended exposure to profound hypothermia and alpha-stat pH management strategy and preexisting developmental delay are associated with the development of choreoathetosis following open-heart surgery 61 in children.

RESPIRATORY SYNCYTIAL VIRUS IMMUNOGLOBULIN AS PROPHYLAXIS AGAINST RESPIRATORY SYNCYTIAL VIRUS IN CHILDREN WITH CONGENITAL HEART DISEASE. • 662

Respiratory Syncytial Virus (RSV) lower respiratory tract infection produces significant morbidity and mortality in young children with congenital heart disease (CHD). Respiratory Syncytial Virus Immunoglobulin (RSVIG) is effective in preventing severe RSV illness in high-risk preterm infants. A 3 year prospective multicenter blinded study was undertaken to determine safety and efficacy of RSVIG as RSV prophylaxis in CHD. Children <48 months were randomized to an infusion group (I) receiving 750 mg/kg RSVIG monthly over the RSV season, or to a control group (C) receiving no RSVIG. Weekly phone surveillance, monthly visits and evaluation of RSV respiratory illness was performed by blinded personnel. Study children included 202 (I) and 214 (C). P=NS for (I) vs (C) for the following variables: mean age [9.3 mo. (I), 10.7 mo. (C)]; gender; crowding; smoke exposure and cardiac severity score but more children with cyanotic CHD were randomized to receive RSVIG [78/202 (I) vs 47/214 (C), p=0.001]. Reduction in RSV hospitalization, ICU admission and mechanical ventilation rates were not statistically significant when the entire group was analyzed. However, in infants <6 months of age at study entry, RSVIG reduced hospitalization by 57% (p=0.01). In acyanotic CHD RSVIG recipients, a trend towards reduced hospital rates and days was also observed[18% (I) vs 10% (C), p=0.06]. Surgically-related severe events (SSE) were more frequent in cyanotic CHD [22/78 (I) vs 4/47 (C), p=0.01] as were surgically-related fatalities [5 (I) vs 0 (C)]. We conclude that RSVIG is safe and should be effective in decreasing serious RSV illness in children <6 months of age with acyanotic CHD. While the increased SSE and surgically-related fatalities appeared to be unrelated to RSVIG administration, children with cyanotic CHD should not be given RSVIG until the cause for this finding has been determined.

Improved Pretransplant Management of Infants with Hypoplastic Left Heart Syndrome Enables Discharge to Home while Waiting for Transplantation

For infants whose families select primary transplantation for hypoplastic left heart syndrome (HLHS), the waiting time averages 3 months. Given the relative shortage of organs, the morbidity and mortality of these patients have been high. Therefore, pretransplant management is critical to improve the number of patients who survive to transplantation. This series shows our evolving management for these children, with an emphasis on nonintensive care. Fifty-two infants with HLHS were listed for primary transplantation at our institution during a 6-year period. The management was aimed at manipulating the pulmonary and systemic blood flows by low-dose continuous infusion of prostaglandin E1 (PGE1), early use of inhaled nitrogen, delayed opening of the atrial septum, and discharge to home with PGE1 infusion for continuing care when the child was on room air and growing. Almost all of the children (46/52) required nitrogen therapy with initial FiO2 of 0.16–0.17. Patients were weaned off nitrogen by 5 to 6 weeks of age. One fourth of the children needed atrial septal opening, typically at 2 or 3 months of age. Seventeen (32.7%) of the infants were able to spend at least some of their waiting time at home. Forty-five of the 52 children (86.5%) survived to receipt of a donor heart. Newborns with HLHS whose families select primary transplantation as their surgical option can be managed with a minimally invasive approach until receipt of a donor heart with an improvement in mortality rate.

Congenital Vertebral-Jugular Fistula in an Infant

SummaryPediatric cardiologists are often consulted to evaluate continuous murmurs in children. This case report describes a child with a congenital vertebral-jugular fistula who presented with torticollis and a continuous murmur. The differential diagnosis of continuous murmurs in this setting is discussed.

157 CARDIAC RHYTHM IN TRANSPOSITION OF THE GREAT ARTERIES PRIOR TO INTRA-ATRIAL SURGICAL CORRECTION

Atrial dysrhythmias and conduction abnormalities are well described after intra-atrial corrective procedures for transposition of the great arteries (TGA). To assess the incidence of such dysrhythmias in children with TGA prior to open heart surgery (OHS), 16 consecutive patients (pts) with this diagnosis underwent 24 hr. ECG monitoring (EM). All 16 pts underwent balloon atrial septostomy (BAS); 3 pts required subsequent surgical atrial septectomy. The initial EMs were performed at a median age of 7 days (Range: 2 days-18 mos.) following clinical stabilization. In 8 pts,EMs were performed both in the newborn period and just prior to OHS. All 24 EMs demonstrated exclusively sinus rhythm with no evidence of atrial dysrhythmias or conduction abnormalities; a single pt manifested occasional unifocal ventricular premature contractions on newborn EM. No change was demonstrated on the serial EMs obtained in the newborn period and prior to OHS. Four pts have undergone a Mustard procedure and 2 a Senning procedure. ECGs obtained 2 mos. to 1 yr. post-operatively now demonstrate low amplitude P waves in 3; a single pt with supra-ventricular tachycardia has frequent atrial premature contractions on EM. In this group of pts with TGA evaluated by EM after BAS and atrial septectomy, cardiac rhythm was normal; dysrhythmias seen after intra-atrial correction appear to be exclusively surgically acquired.

161 PULMONARY ARTERY BANDING AS PRIMARY THERAPY FOR COMPLETE ATRIO-VENTRICULAR CANAL

Due to the high mortality of early primary correction twenty-five children with complete atrio-ventricular canal less than one year of age have had pulmonary artery (PA) banding during the last twelve years. All patients had congestive heart failure with PA pressure equal to systemic, and a large ventricular septal defect on angiography. Banding was performed at 7 weeks to 11 months of age. Nineteen were less than 6 months of age at surgery. Seventeen of 25 had Trisomy 21.Early mortality (under 30 days) was 20% and total mortality 40% for the series. Three of the older survivors have had open correction, two successfully. Since 1973 there has been 92% early survival (11/12) and 83% total survival. During this period PA and aortic pressures have been measured post-banding in the operating room with PA pressure aimed at 50% of systemic. Banding has been performed without regard to the angiographic degree of mitral regurgitation (MR). Six of 9 patients without MR have survived, but 6 of 9 with moderate or severe MR have also survived. Degree of MR also failed to correlate with age of presentation.Pulmonary artery banding is effective palliation for infants with congestive heart failure due to complete atrio-ventricular canal when a large ventricular component causes systemic PA pressure. Intraoperative pressure measurements are useful to determine the gradient from banding. Mitral regurgitation was not a factor in the success of banding.

PROSPECTIVE DEVELOPMENT OF A CARDIAC SCORING SYSTEM TO PREDICT THE SEVERITY AND OUTCOME OF CONGENITAL HEART DISEASE (CHD). 211

There is a pressing need for a simple and reproducible cardiac scoring system (CSS) to predict the severity of CHD. We have previously developed a CSS based on the published risk of common congenital cardiac defects. Components of the CSS are scored as follows: cyanosis (0-room air saturation>85%, 1-saturation 2/3 systemic), and heart failure (0-none, 1-controlled with medication, 2-uncontrolled). The total score ranges from 0-6. To assess the usefulness of the CSS, 416 children with all types of CHD(age < 48 months) enrolled in a prospective trial of high titer RSV immune globulin to prevent RSV illness were scored on entry. Patients were enrolled over a three year period at 17 centers and were followed for 8 months(December through August) in the year they were entered. Score at entry: 0(n=25), 1(n=121), 2(n=141), 3(n=92), 4(n=28), 5(n=7), not scored (n=2) was assessed against other measures of morbidity and mortality. This CSS predicted hospitalization, need for cardiac surgery, and mortality in a large population of children with multiple forms of CHD. We propose that this CSS is able to predict both morbidity and mortality in children with simple and complex CHD.Table

PRIMARY PULMONARY HYPERTENSION IN PEDIATRIC PATIENTS AT ALTITUDE

Five patients (4-9 yrs.) presented in five years with primary pulmonary hypertension (PPH). Each was initially evaluated for seizures or syncope. Two sisters (#1s2) were Hispanic and resided at 5,200′. Two brothers (#3&4) were black and resided at 5,200′. An only child (#5) resided at 9,000′.Cardiac catheterization with hyperoxia was performed in all and one received additional prostaglandin 12 (PGI2). Structural heart disease was ruled out in all. Resistance ratios (R.R.) were calculated in room air and 100% O2.Three of the five patients died 9.2 months after diagnosis. Two of the deaths (#1&2) occurred after steady clinical deterioration at 5,200′. The third child (#5) dramatically improved after moving to sea level but died five days after returning to altitude. One of the black males has significantly improved since moving to sea level (#4) and his brother with milder disease has spontaneously improved while remaining at altitude (#3).PPH is a rare and rapidly fatal disease in childhood. In patients presenting at altitude, the clinical course may be altered or reversed by moving to sea level.

111 HEMODYNAMIC ASSESSMENT OF PULMONARY INSUFFICIENCY AFTER TETRALOGY OF FALLOT REPAIR

Pulmonary insufficiency (PI) has been implicated in the etiology of VPCs and biventricular dysfunction after repair of Tetralogy of Fallot (TF). To assess this, 50 consecutive postop TF patients with clinical PI were evaluated prospectively with branch pulmonary artery (PA) angiography at follow-up catheterization. Group I consisted of 23 pts who cleared contrast from the right heart normally, within 2 beats of injection; Group II consisted of 27 pts who required 3-12 beats to clear the right heart (x=6). Groups did not differ significantly in age at surgery or evaluation, or in residual RV hypertension. They were compared for RV size by M-mode echocardiography using RV/LV diastolic dimension ratio, for RV and LV ejection fractions (EF) by radionuclide ventriculography, and for grade of VPCs on treadmill exercise and/or ambulatory EGG. Findings are summarized below:In this study, prolonged right heart opacification after branch PA injection correlated significantly with RV dilatation, biventricular dysfunction and high grade ventricular ectopy. After TF repair, branch PA angiography provides a useful adjunctive method of assessing the variable impact of pulmonary insufficiency.