*****AMERICAN HEART JOURNAL*****

(REFERENCE 1 OF 8) 99158571

Hoshino K Ogawa K Hishitani T Kitazawa R Uehara R Hypoplastic left heart syndrome: duration of survival without surgical intervention.

In: Am Heart J (1999 Mar) 137(3):535-42

ISSN: 0002-8703

BACKGROUND: Mortality rate for heart transplantation for patients with hypoplastic left heart syndrome (HLHS) has improved, but there is a considerable wait until a suitable donor is available. Thus it is important to examine the duration of survival and risk factors for early death in patients with HLHS who did not undergo surgical intervention. METHODS AND RESULTS: Twenty-six consecutive patients were studied retrospectively. Duration of survival and the 14 following variables were investigated: date of birth, body weight at birth, cardiothoracic ratio, ascending aorta diameter, interatrial communication size, coarctation of the aorta, tricuspid regurgitation, anatomic subtype (patency) of mitral and aortic valve, arterial blood gas findings (pH, PaO 2, SaO 2, PaCO2, base excess), and ST depression in the electrocardiogram. Twenty patients survived <60 days (group A) and 6 patients survived beyond 60 days (group B). The duration of survival (mean [SD]) was 60 (151) days overall (1 patient is currently alive at 783 days). The long-term survivors (beyond 60 days) increased significantly after 1991 (P <.05). Coarctation of the aorta was a significant risk of early death (<60 days) (P <.05). Interatrial communication size was significantly smaller in group B than in group A (P <.05). The mean pH and base excess were significantly lower in group A than in group B. The other 9 variables showed no significant difference between the 2 groups. CONCLUSIONS: There was a significant correlation of long-term survival with stabilized ductal blood flow without coarctation of the aorta, adequate restriction of interatrial communication without severe hypoxemia, and no metabolic acidosis.

Registry Numbers: 124-38-9 (Carbon Dioxide) 7782-44-7 (Oxygen)

Institutional address: Department of Pediatric Cardiology Saitama Children's Medical Center Iwatsuki City Saitama Japan.

*****AMERICAN JOURNAL OF CARDIOLOGY*****

(REFERENCE 2 OF 8) 97373671

Kern JH Hayes CJ Michler RE Gersony WM Quaegebeur JM Survival and risk factor analysis for the Norwood procedure for hypoplastic left heart syndrome.

In: Am J Cardiol (1997 Jul 15) 80(2):170-4

ISSN: 0002-9149

The optimal approach to hypoplastic left heart syndrome (HLHS) is controversial. The palliative Norwood operation, cardiac transplantation, and no surgical intervention have all been advocated. Centers that perform the Norwood operation have met with varied results, and conflicting reports exist regarding factors predictive of stage I outcome. From January 1990 to January 1996, 67 patients with HLHS were admitted with intent to perform the staged Norwood procedure. Fourteen patients did not undergo surgery. In the 53 patients treated surgically, outcome was reviewed, and 10 potential risk factors for first stage mortality were analyzed. Forty- one infants survived the Norwood I operation to hospital discharge (77% of the surgically treated patients and 61% of the entire group, including those who did not undergo operation) with 6 additional deaths 3 to 5 months after operation. Univariate analysis showed cardiopulmonary bypass time and circulatory arrest time to be significant risk factors for hospital mortality. Multivariate analysis revealed only cardiopulmonary bypass time as significant (p <0.01). Of the 15 prenatally diagnosed newborns who underwent surgery, 11 survived (p = 0.72). Ten of 11 patients with preoperative organ damage survived (p = 0.42). Among the 35 bidirectional Glenn (Norwood II) and Fontan (Norwood III) procedures performed, there were 2 deaths. The 5-year actuarial survival for patients who underwent operations was 61%. The Norwood procedure is a favorable option for the infant with HLHS. Surgical survival may be affected by a prolonged cardiopulmonary bypass time, but is not affected by other factors analyzed, including prenatal diagnosis and preoperative organ damage.

Institutional address: Department of Pediatrics Columbia-Presbyterian Medical Center Columbia University College of Physicians and Surgeons New York New York 10021 USA.

*****ANNALS OF THORACIC SURGERY*****

(REFERENCE 3 OF 8) 95194088

Ibrahim M Masters RG Hendry PJ Davies RA Smith S Struthers C Walley VM Keon WJ Determinants of hospital survival after cardiac transplantation.

In: Ann Thorac Surg (1995 Mar) 59(3):604-8

ISSN: 0003-4975

To identify the preoperative factors that influence hospital survival after transplantation we analyzed our consecutive experience of 183 transplantations in 179 patients over a 10-year period. There were 151 male and 29 female transplant recipients ranging in age from 10 days to 70 years (mean, 48 +/- 1 years). Diagnoses included coronary disease in 110 patients, cardiomyopathy in 55 patients, valvular disease in 6 patients, and congenital heart disease in 9 patients. Seventy-seven had undergone a previous cardiac operation, and 30 patients required preoperative mechanical support. Forty patients received hearts from donors who were 40 years old or older (range, 40 to 62 years). Ischemic time was greater than 240 minutes in 32 cases, and pulmonary vascular resistance was greater than 3 Wood units in 40 patients (range, 3.1 to 10.0 Wood units). Cyclosporine induction was used in 52 patients, whereas 128 recipients received polyclonal antibody prophylaxis. There were 25 hospital deaths. Recipient diagnosis, use of mechanical support, donor age, and the immune suppression protocol were related to hospital survival according to univariate analysis. Using multiple logistic regression, only the method of immune suppression induction and the use of mechanical assists were significant independent determinants of survival. In conclusion, we believe that extended ischemic times and donor age do not adversely affect the early success of transplantation, whereas induction with immune globulin may reduce early mortality. Patients requiring mechanical support before transplantation continue to be a challenge.

Institutional address: University of Ottawa Heart Institute Division of Cardiac Surgery Ottawa Civic Hospital Canada.

*****CIRCULATION*****

(REFERENCE 4 OF 8) 94037409

Bando K Konishi H Komatsu K Fricker FJ del Nido PJ Francalancia NA Hardesty RL Griffith BP Armitage JM Improved survival following pediatric cardiac transplantation in high- risk patients.

In: Circulation (1993 Nov) 88(5 Pt 2):II218-23

ISSN: 0009-7322

BACKGROUND. Preoperative hemodynamic support, complex congenital heart disease, and elevated pulmonary vascular resistance present particular challenges for pediatric heart transplantation. This study was performed to identify preoperative factors that influence survival after pediatric heart transplantation over two eras of pediatric heart transplant experience. METHODS AND RESULTS. We retrospectively analyzed demographic, clinical, and hemodynamic data from 67 pediatric patients who underwent heart transplantation between February 1982 and June 1992 and compared survival between two eras (early experience versus late experience). During the early experience (group 1: February 1982 to August 1989), univariate analysis identified congenital heart disease, pretransplant extracorporeal membrane oxygenator (ECMO) support, inotropic and/or ventilatory support (UNOS status I), elevated transpulmonary gradient (at least 15 mm Hg), and elevated pulmonary vascular resistance index (at least 4 Wood units.m2) as preoperative risk factors for early death after pediatric heart transplantation. However, in the late experience (group 2: September 1989 to June 1992), the only risk factor for premature death by univariate analysis was elevated transpulmonary gradient. By multivariate analysis, elevated transpulmonary gradient was the only risk factor for our early, late, and entire experiences. One-year survival after transplantation for congenital heart disease was improved from 46% in group 1 to 73% in group 2 (P < .05). In group 1, only one patient (25%) with pretransplant ECMO support survived 1 year, whereas 66% (four of six) survived more than 1 year in group 2. CONCLUSIONS. Although elevated transpulmonary gradient continues to be a significant risk factor for pediatric heart transplantation, candidates with congenital heart disease, UNOS status I, and pretransplant ECMO support now can be successfully transplanted with reasonable hope for extended survival.

Institutional address: Division of Cardiothoracic Surgery University of Pittsburgh Pa.

*****JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY*****

(REFERENCE 5 OF 8) 96393342

Cetta F Feldt RH O'Leary PW Mair DD Warnes CA Driscoll DJ Hagler DJ Porter CJ Offord KP Schaff HV Puga FJ Danielson GK Improved early morbidity and mortality after Fontan operation: the Mayo Clinic experience, 1987 to 1992.

In: J Am Coll Cardiol (1996 Aug) 28(2):480-6

ISSN: 0735-1097

OBJECTIVES: This study sought to evaluate changes in early morbidity and mortality as well as predictors of outcome in our most recent 339 patients undergoing modified Fontan operations. BACKGROUND: The Fontan operation is the preferred definitive palliation for patients with functional single ventricles. Previously reported early mortality rates after Fontan operation have been substantial. METHODS: Records of 339 consecutive patients who had a Fontan operation at the Mayo Clinic between 1987 and 1992 (recent cohort) were reviewed. This cohort was compared with the previous 500 patients who had Fontan operations performed between 1973 and 1986 (early cohort). RESULTS: Recently, overall early mortality after Fontan has decreased significantly compared with that for the early cohort (from 16% to 9%, p = 0.002). This decline occurred despite increased anatomic complexity of patients. Short-term posthospital survival has also improved significantly in recent patients. One-year survival improved to 88% from 79%, and 5-year survival to 81% from 73% (p = 0.006). Patients with common atrioventricular valves and those who took daily preoperative diuretic medication or had either postoperative renal failure or elevated postbypass right atrial pressure were at increased risk for early mortality. Young age was not found to be a risk factor for early mortality. Early mortality for patients with heterotaxia decreased dramatically: recent 30-day mortality was 15% compared with 41% in the early heterotaxy cohort. CONCLUSIONS: Many factors may have contributed to decreased early mortality after Fontan. Improved patient selection, younger age at time of operation, refinements in surgical techniques and postoperative management may all have had important roles. Proposed technical modifications of the Fontan operation must be evaluated in light of these improved results.

Institutional address:

Section of Pediatric Cardiology Mayo Clinic Rochester Minnesota 55905 USA.

*****JOURNAL OF PEDIATRICS*****

(REFERENCE 6 OF 8) 90133210

Pahl E Fricker FJ Armitage J Griffith BP Taylor S Uretsky BF Beerman LB Zuberbuhler JR Coronary arteriosclerosis in pediatric heart transplant survivors: limitation of long-term survival.

In: J Pediatr (1990 Feb) 116(2):177-83

ISSN: 0022-3476

Because coronary atherosclerosis after heart transplantation has been a limiting problem in long-term survival of adults, we reviewed the coronary angiograms, and autopsy data when available, from 21 of 30 children who underwent orthotopic heart transplantation and survived the perioperative period. Six patients had coronary atherosclerosis, and five of these patients died 6 months to 3 years after heart transplantation. The late deaths were sudden and unexpected. Coronary angiography demonstrated several types of lesions, including concentric narrowing, tubular segmental lesions, and abrupt obliteration of major coronary vessels. Risk factors assessed included hypertension, hyperlipidemia, cytomegalovirus infection, type of immunosuppressive regimen, number of rejection episodes, and major histocompatibility antigen mismatches. Only the frequency and duration of rejection episodes seemed to be more prevalent in the patients in whom coronary atherosclerosis developed. Despite the benefits of heart transplantation in treating children with end-stage heart disease, coronary atherosclerosis may limit long-term survival. We suggest that these children should undergo serial coronary angiography to identify those at risk for subsequent events related to coronary artery disease.

Institutional address: Department of Pediatrics Pathology and Cardiothoracic Surgery Children's Hospital of Pittsburgh Pennsylvania.

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(REFERENCE 7 OF 8) 98171720

Johnston JK Chinnock RE Zuppan CW Razzouk AJ Gundry SR Bailey LL Limitations to survival for infants with hypoplastic left heart syndrome before and after transplant: the Loma Linda experience.

In: J Transpl Coord (1997 Dec) 7(4):180-4; quiz 185-6

ISSN: 0905-9199

Untreated, hypoplastic left heart syndrome is a lethal cardiac defect. Heart transplant has become an accepted therapeutic option for this condition. However, significant limitations to survival remain for infants with this condition who are referred for heart transplantation. Attention to the prevention, early detection, and management of common problems occurring at each stage of the transplantation process is important for improving survival rates. This study retrospectively reviewed the cases of 195 infants with hypoplastic left heart syndrome registered for heart transplantation at Loma Linda University Medical Center between November 1985 and July 1996 to determine causes of death. During the waiting period, progressive cardiac failure and complications from interventional procedures were the leading causes. In the early postoperative period, technical issues and acute graft failure were most important, whereas late deaths (more than 30 days after transplant) were most often related to rejection and posttransplant coronary artery disease.

Institutional address: Loma Linda University Medical Center Calif. USA.

*****LANCET*****

(REFERENCE 8 OF 8) 91278617

Ruiz MJ Lopez-Herce J Tamariz A Garcia-Teresa MA Long survival in hypoplastic left heart syndrome [letter; comment]

In: Lancet (1991 Jul 6) 338(8758):53

ISSN: 0140-6736

[No Abstract Available]

Comment on: Lancet 1991 Apr 20;337(8747):957-9