Hypoplastic left heart syndrome diagnosed in fetal life: associated findings, pregnancy outcome and results of palliative surgery

Galindo, Alberto; Nieto, Olga; Villagrá, S.; Grañeras, A.; Herraı̀z, Ignacio; Mendoza, Alberto

doi:10.1002/uog.6355

Cited by 47 publications

(43 citation statements)

References 41 publications

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“…Operative survival reported from pediatric studies ranged widely from 40 to 61% [2][3][4] , but some centers reported survival rates approaching 90% [5,6] . However, fetal series generally showed much lower survival figures, usually below 50% [7][8][9] , and HLHS was mostly included at the worst end of the severity spectrum [10] .…”

mentioning

confidence: 99%

Fetal Aortic Valvuloplasty: Experience and Results of Two Tertiary Centers in Spain

et al. 2017

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Objective: Fetal aortic valvuloplasty (FAV) may avoid progression of critical aortic stenosis (CAS) to hypoplastic left ventricle, improving the options for biventricular circulation (BVC). We describe the results of FAV in 2 referral centers in Spain. Methods: We analyzed all FAVs performed in the period 2007-2015. The selection of candidates, the technique, and postnatal management were made following an agreed protocol. A descriptive analysis of survival, type of circulation after birth, and complications was made, considering all deaths in the first 48 h after FAV as FAV-related. Results: FAV was performed in 28 fetuses at a median gestational age (GA) of 23 weeks (range, 20-32). FAV was technically successful in 22 (78.6%), of whom 11 were born alive and with intention to treat. Eight (72.7%) resulted in BVC and 3 (27.3%) in univentricular circulation. The rate of FAV-related deaths was 32%. These patients underwent FAV earlier than live-born fetuses (median GA at FAV 22 weeks [range, 20.0-25.0] vs. 24.5 weeks [range, 21.0-32.0], respectively, p = 0.031). Conclusions: A significant proportion of fetuses with CAS who undergo technically successful FAV have BVC postnatally. However, FAV implies a high risk of fetal death, which highly depends on the GA at which this intervention is required.

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confidence: 99%

Fetal Aortic Valvuloplasty: Experience and Results of Two Tertiary Centers in Spain

et al. 2017

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“…These limitations worsen with the negative feedback generated by the high rate of termination of pregnancy (TOP) following prenatal diagnosis of HLHS. [7] With the incidence of HLHS about 0.1-0.25/1000 live births, in the Indian scenario, we should expect about 2000 babies with HLHS born every year. In reality, the total number of babies with HLHS seen in major centers in India will not be more than 100 children/year.…”

Section: Discussionmentioning

confidence: 99%

“…79% (58/73) of the cases in which HLHS was detected at ≤22 weeks were terminated. [7] HLHS constitutes 5% of all cases of congenital heart disease and is responsible for 25% of cardiac deaths in the 1st week of life. Among 10,000 live births, approximately 1.8 will be born with HLHS, with a slight male predominance.…”

Section: Discussionmentioning

confidence: 99%

Hypoplastic left heart syndrome with parchment left ventricle: A rare perinatal autopsy case report

Chatura¹,

Neethu²,

Mavintop³

2015

JMRPS

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Hypoplastic left heart syndrome (HLHS) refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood fl ow from the left ventricular outfl ow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. This is a fetal autopsy case report of HLHS, which was diagnosed on ultrasonography and medically terminated at 20 weeks of gestation. The autopsy and histopathological fi ndings are discussed in detail. Some important aspects of epidemiology, prenatal diagnosis, and current literature on HLHS are highlighted.

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“…The delay in diagnosis, I n turn, may lead to systemic hypoperfusion, shock, and multiorgan damage, which can diminish chances for surgical success and lead to long-term sequelae. [4] Double outlet right ventricle is characterized by the aorta and the pulmonary trunk both arising from the right ventricle. This anomaly accounts for 2% of live births with a congenital heart defect.…”

Section: Introductionmentioning

confidence: 99%

Prenatal Imaging of Congenital Fetal Anomalies: A Case Series

Ravikanth¹,

Sarkar²,

Kumar³

et al. 2017

AIMDR

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Hypoplastic left heart syndrome (HLHS) comprises 2 to 3 percent of all congenital heart disease. HLHS is uniformly lethal if left untreated; however, there is currently an estimated 70 percent 5-year survival rate for infants who undergo surgical correction. The hypoplastic left heart syndrome encompasses a spectrum of cardiac malformations that are characterized by significant underdevelopment of the components of the left heart and the aorta, including the left ventricular cavity and mass. Its main anatomical characteristics include mitral stenosis or atresia, aortic stenosis or atresia, and hypoplasia of left ventricle. The apex of the heart is usually formed by the right ventricle. The right ventricle is the single functional ventricle maintaining fetal circulation in these patients.After births, due to decreased pulmonary vascular resistance and closure of the ductus arteriosus, infants with HLHS develop a constellation of symptoms that include cyanosis, dyspnea, heart failure, hypoxemia and acidosis. Surgery is the only effective treatment. Unfortunately, even when treated, this disease is associated with significant morbidity including an increased risk of thrombotic complications, decrease in exercise tolerance, and neuro-developmental impairment. Continuous intravenous prostaglandin E1 is used to maintain a PDA to allow adequate systemic perfusion. Nitrogen may be used in some centers to decrease inspired oxygen which increases pulmonary arterial resistance. This therapy encourages systemic rather than pulmonary blood flow and enhances systemic perfusion. Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both the aorta and pulmonary artery arise entirely or predominantly from the right ventricle (RV). The only outlet from the left ventricle (LV) is a ventricular septal defect (VSD). DORV is usually associated with concordant atrioventricular connections with the right atrium draining into the RV and the left atrium draining into the LV. DORV is almost always associated with a VSD and occasionally with an atrial septal defect. Transposition of great arteries is characterized by both the outflows being parallel to each other at the origin. On color Doppler, they show similar color flows, indicating that the direction of flow in both the great vessels is same. Most atrial septal defects involve either the septum primum or septum secundum. Primum atrial septal defect is the simplest form of the atrioventricular septal defect. Secundum atrial septal defect which is the commonest are usually isolated septal defects, but may be related to other cardiac anomalies like mitral, tricuspid, pulmonary or aortic atresia and are occasionally found as part of syndromes like Holt-Oram syndrome.

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Hypoplastic left heart syndrome diagnosed in fetal life: associated findings, pregnancy outcome and results of palliative surgery

Abstract: Objectives To analyze the main prenatal characteristics of hypoplastic left heart syndrome (HLHS), its association with extracardiac anomalies including increased nuchal translucency (NT) and the outcome of affected patients. Methods

Cited by 47 publications

References 41 publications

Fetal Aortic Valvuloplasty: Experience and Results of Two Tertiary Centers in Spain

Fetal Aortic Valvuloplasty: Experience and Results of Two Tertiary Centers in Spain

Hypoplastic left heart syndrome with parchment left ventricle: A rare perinatal autopsy case report

Prenatal Imaging of Congenital Fetal Anomalies: A Case Series

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