Hypoplastic left heart syndrome: Duration of survival without surgical intervention

doi:10.1016/S0002-8703(99)70503-X

American Heart Journal

Volume 137, Issue 3, March 1999, Pages 535-542

https://doi.org/10.1016/S0002-8703(99)70503-X Get rights and content

Abstract

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, PaO₂ , SaO₂ , 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. (Am Heart J 1999;137:535-42.)

Section snippets

Patients

The medical records of 41 consecutive patients seen at Saitama Children’s Medical Center with HLHS between April 1983 and March 1996 were reviewed retrospectively, including echocardiographic records, electrocardiographic records, and hospital charts. The diagnosis of HLHS was based on 2-dimensional echocardiographic evidence of a diminutive ascending aorta, aortic atresia, or stenosis and a hypoplastic left ventricle.⁸ Patients with associated atrioventricular septal defect (malaligned) were

Outcome and duration of survival

Fig 1 summarizes the outcome for all 38 patients seen with HLHS.

. Outcome of 38 consecutive patients seen at Saitama Children’s Medical Center with HLHS from April 1983 to March 1996. Ao anast, Side-to-side anastomosis of ascending aorta to pulmonary artery; PAB, pulmonary artery banding.

Of the 12 patients undergoing palliative surgery, 10 died at a median of 33 days (range 0 to 216 days) after Norwood operation, and another underwent anastomosis of ascending aorta to pulmonary artery and

Discussion

Previously, HLHS was considered uniformly fatal, but recently the outlook has dramatically improved. Increasing experience with both reconstructive palliative surgery and heart transplantation has resulted in significant improvement of survival.8, 11, 12 However, as for heart transplantation, donor availability is a major determinant of applicability, and there is a considerable wait until a suitable donor is available. Thus it is important to define the duration of survival and risk factors

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Cited by (21)

Rapid bilateral pulmonary artery banding: A developmentally based proposal for the management of neonates with hypoplastic left heart
2023, JTCVS Open
Hypoplastic Left Heart Syndrome: Parent Support for Early Decision Making
2013, Journal of Pediatric Nursing
Citation Excerpt :
Once HLHS is detected after birth, intravenous prostaglandins are initiated at 0.05 to 0.1 Hg/kg per minute in order to maintain the patency of the ductus arteriosis, enabling blood flow to the aorta (Soetenga & Mussatto, 2004). If the child's condition deteriorates either due to heart failure, vascular collapse, or side effects of prostaglandins, the following interventions are initiated; mechanical ventilation, inotropic support, bicarbonate infusion, diuretic infusion, and sedatives to treat cardiac and respiratory insufficiency (Hoshini, Ogawa, Hishitani, Kitazawa, & Vehara, 1999; Soetenga & Mussatto, 2004). Rarely, some HLHS infants will have severe hypoxemia due to a severely restrictive or non-communicating atrium.
Hypoplastic left heart syndrome is a life threatening congenital cardiac anomaly. After a child has been diagnosed with hypoplastic left heart syndrome, parents must make life or death decisions within days of birth. Healthcare providers must provide appropriate education so that parents are able to make informed, timely decisions. Information regarding the diagnosis, treatment options, and parental decision making process for initial decision making for hypoplastic left heart syndrome are provided to guide nurses who work with these families. The challenging decision making process that parents must go through after diagnosis of hypoplastic left heart syndrome will be described.
The prevalence of adult congenital heart disease, results from a systematic review and evidence based calculation
2012, American Heart Journal
The prevalence of adult patients with congenital heart disease (CHD) has been reported with a high degree of variability. Prevalence estimates have been calculated using birth rate, birth prevalence, and assumed survival and derived from large administrative databases. To report more robust prevalence estimate, we performed a systematic review for studies concerning CHD prevalence in adults. Moreover, to diminish bias of calculated estimates, we conducted an evidence-based calculation for the Netherlands.
A systematic database search was performed to identify reports on the prevalence of adult CHD. Bicuspid aortic valve, mitral valve prolapse, Marfan syndrome, cardiomyopathy, congenital arrhythmia, and spontaneously closed defects were excluded. In addition, CHD prevalence was calculated using birth rate, birth prevalence, and survival estimates.
Our search yielded 10 publications on the prevalence of CHD in adults. Four reported results from population wide cross-sectional data, whereas in 6, prevalence was calculated. Mean prevalence reported by empirical studies was 3,562 per million when unspecified lesions were included and 2,297 per million when these were excluded. Mean prevalence derived from calculation was 3,536. Our calculated estimate was 3,228 per million adults. Taking these estimates as well as the limitations inherent to their derivation into consideration, the prevalence of CHD in the adult population is approximately 3,000 per million adults.
This systematic review presents a comprehensive overview of publications on the prevalence of CHD in adults. The best available evidence suggests that overall prevalence of CHD in the adult population is in the region of 3,000 per million.
Decreasing Number of Deaths of Infants with Hypoplastic Left Heart Syndrome
2008, Journal of Pediatrics
To assess mortality rates and demographic characteristics for infants with hypoplastic left heart syndrome (HLHS) in California.
We used California death registry files from 1990 to 2004 to compare overall mortality and demographic characteristics between infants with HLHS (n = 856) who received surgical intervention and those who received comfort care. The California discharge database was used to calculate the annual incidence of disease and survival rates for infants with HLHS undergoing surgery between 1995 and 1999.
The annual number of deaths for infant with HLHS decreased by nearly 50% over the study period, even though the incidence of the disease remained constant during this period. For all deaths, the proportion of infants receiving comfort care decreased significantly over time compared with those infants who underwent surgery. Although the total number of deaths in infants with HLHS who underwent surgical intervention increased, the mortality rate for this cohort decreased. Interstage unexpected mortality and the median age at death both increased in the infants who underwent surgery.
Over the study period of 1990 to 2004 in California, fewer families chose comfort care for infants diagnosed with HLHS, and the number of deaths for those infants who underwent surgical intervention increased. These changes likely reflect improved treatment outcomes and an increased number of families desiring surgical intervention in higher-risk infants.
Impaired Power Output and Cardiac Index With Hypoplastic Left Heart Syndrome: A Magnetic Resonance Imaging Study
2006, Annals of Thoracic Surgery
Citation Excerpt :
The non-HLHS group seemed to have a higher stroke index compared with the HLHS group, although this was not statistically significant. Hypoplastic left heart syndrome is one of the most complex and lethal forms of single-ventricle diseases, and its management is critical at the neonatal stage [3, 5, 15–25]. With the introduction of the staged reconstruction process and improved diagnosis of HLHS by fetal echocardiography, the early survival rate has markedly improved [4, 24].
Unfavorable cardiac mechanics in children with hypoplastic left heart syndrome (HLHS) when compared with other single-ventricle defects may affect long-term morbidity and outcome. Using noninvasive phase contrast magnetic resonance imaging (PC MRI), we examined cardiac mechanics in children with HLHS and compared the results to other single-ventricle defects.
Eighteen children with HLHS and 18 children with other single-ventricle defects were studied after the Fontan operation. Phase contrast MRI scans were obtained perpendicular to the ascending aorta, and flow was quantified using an in-house segmentation and reconstruction scheme. The total power output was determined using the modified Bernoulli equation along with cardiac output and systemic vascular resistance index.
Compared with non-HLHS congenital heart defects, children with HLHS had significantly lower power output (1.40 ± 0.39 versus 1.78 ± 0.38 W/m², p < 0.004) and cardiac index (3.15 ± 0.97 versus 4.09 ± 1.23 L · Min⁻¹ · m⁻², p < 0.009) with a concomitant higher systemic vascular resistance index (28.94 ± 11.5 versus 22.7 ± 8.53 WU, p < 0.03) despite generating similar systolic blood pressures (112.9 ± 22.4 versus 115.2 ± 23 mm Hg, p > 0.05).
Minimally invasive measurements with PC MRI in children with HLHS showed significantly lower power output and cardiac index when compared with other single-ventricle physiologies. Abnormal aortic flow patterns may contribute to power loss and may have long-term survival and morbidity implications associated with the Fontan procedure. Elevated systemic vascular resistance index despite similar blood pressure opens avenues for therapeutic intervention for afterload reduction.
Ethical considerations in the management of hypoplastic left heart syndrome
2002, Newborn and Infant Nursing Reviews

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^☆: Reprint requests: Kenji Hoshino, MD, Department of Pediatric Cardiology, Saitama Children’s Medical Center, 2100 Magome, Iwatsuki City, Saitama, Japan-339.

^☆☆: 0002-8703/99/$8.00 + 0 4/1/87942

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Hypoplastic left heart syndrome: Duration of survival without surgical intervention☆,☆☆

Abstract

Section snippets

Patients

Outcome and duration of survival

Discussion

Lancet

Ann Thorac Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

Am Heart J

J Am Coll Cardiol

J Am Coll Cardiol