Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia: an Algorithmic Approach

Balduf, Kaitlin; Kumar, T.K. Susheel; Boston, Umar S.; Sathanandam, Shyam; Lee, Marc V.; Jancelewicz, Timothy; Knott‐Craig, Christopher J.

doi:10.1053/j.semtcvs.2018.02.010

Cited by 10 publications

(3 citation statements)

References 18 publications

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“…Previous reports described mechanical ventilation at the time of, and following correction of congenital heart defects, can worsen CLE [4]. This is best exemplified by mortality rates of 40-100% seen in patients with HLHS and CLM, particularly congenital diaphragmatic hernia [5]. Some reports emphasized the importance of evaluating for CLE in setting of congenital heart disease to prevent prolonged respiratory distress and ventilation dependence in postoperative period, and have debated about management options for children with CLE and congenital heart diseaseto address cardiac versus pulmonary defects first, or both simultaneously [4,6].…”

Section: Discussionmentioning

confidence: 99%

Surgical palliation for an infant with hypoplastic left heart syndrome and congenital lobar emphysema: a case report

Sahulee¹

2021

JCRR

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Hypoplastic left heart syndrome is a form of congenital heart disease that is associated with significant lifelong morbidity and mortality. Although rarely reported together, the prognosis for patients with hypoplastic left heart syndrome and congenital anomalies of the airway or lung, such as congenital diaphragmatic hernia, has been shown to be very poor. The outcomes for patients with hypoplastic left heart syndrome and congenital lobar emphysema specifically is currently unknown. Reported herein is the first case of a child with a rare combination of hypoplastic left heart syndrome and congenital lobar emphysema, and a discussion of his presentation, diagnosis, management and outcome to date.

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Section: Discussionmentioning

confidence: 99%

Surgical palliation for an infant with hypoplastic left heart syndrome and congenital lobar emphysema: a case report

Sahulee¹

2021

JCRR

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“…HLHS is the second most common congenital heart disease associated with congenital diaphragmatic hernia. The reported survival rate of neonates with congenital diaphragmatic hernia and HLHS is only 1–5% [ 17 ].…”

Section: Discussionmentioning

confidence: 99%

Case Report: 5 Cases of Variant Hypoplastic Left Heart Syndrome Diagnosed on Prenatal Fetal Ultrasound

Pribadi,

Siddiq,

Nugrahani

et al. 2023

Am J Case Rep

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Case series Patients: Female, 38-year-old • Female, 41-year-old • Female, 36-year-old • Female, 43-year-old • Female, 30-year-old Final Diagnosis: Hypoplastic left heart syndrome Symptoms: Heart failure Clinical Procedure: — Specialty: Obstetrics and Gynecology Objective: Congenital defects/diseases Background: Hypoplastic left heart syndrome (HLHS) is a complex left-sided obstructive congenital cardiac condition with several variants. This report is of 5 cases with varying morphology of HLHS diagnosed by fetal prenatal ultra-sound at the 4-chamber view (4CV) level. Case Reports: Five cases were referred by obstetrics and gynecology specialists with preliminary information on visible congenital abnormalities in the third trimester. Fetal echocardiography showed that several morphological variants of HLHS were found. The patient in the first case had the most frequently found variant; this variation is usually linked to mitral valve stenosis (MVS). The second case had the characteristic of mitral valve atresia (MVA), and as a consequence, blood flow was not visible in this variant and the left ventricle (LV) was not clear or not adequately visualized by ultrasound. In the third case, the patient suffered from severe MVS and perhaps a small aorta. Uni-atrial conditions were described in the third case. In the fourth case, the patient had a narrow LV, MVA, ventricular septal defect, nearly united atrium, and tricuspid regurgitation. The fifth case was a case of HLHS with congenital diaphragmatic hernia. Further, 4 out of 5 of the cases were associated with widening of the cisterna magna and Dandy Walker syndrome-associated posterior fossa disorder malformations. The overall maternal age was over 35 years old in 4 cases. Karyotyping examination was not performed in all cases. Conclusions: The role of ultrasound is very important in determining the diagnosis and the degree of development of hypo-plastic LV. However, visualization at the 4CV level can detect abnormalities found in the LV.

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“…Though no formal guidelines exist for management of CHD in CDH, an algorithmic, team approach has been advocated to assist in complex decision-making for critical lesions ( 11 ).…”

Section: The Fetal Heart In Congenital Diaphragmatic Herniamentioning

confidence: 99%

The heart in congenital diaphragmatic hernia: Knowns, unknowns, and future priorities

Patel

Massolo²,

Kraemer³

et al. 2022

Front. Pediatr.

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There is growing recognition that the heart is a key contributor to the pathophysiology of congenital diaphragmatic hernia (CDH), in conjunction with developmental abnormalities of the lung and pulmonary vasculature. Investigations to date have demonstrated altered fetal cardiac morphology, notably relative hypoplasia of the fetal left heart, as well as early postnatal right and left ventricular dysfunction which appears to be independently associated with adverse outcomes. However, many more unknowns remain, not least an understanding of the genetic and cellular basis for cardiac dysplasia and dysfunction in CDH, the relationship between fetal, postnatal and long-term cardiac function, and the impact on other parts of the body especially the developing brain. Consensus on how to measure and classify cardiac function and pulmonary hypertension in CDH is also required, potentially using both non-invasive imaging and biomarkers. This may allow routine assessment of the relative contribution of cardiac dysfunction to individual patient pathophysiological phenotype and enable better, individualized therapeutic strategies incorporating targeted use of fetal therapies, cardiac pharmacotherapies, and extra-corporeal membrane oxygenation (ECMO). Collaborative, multi-model approaches are now required to explore these unknowns and fully appreciate the role of the heart in CDH.

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Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia: an Algorithmic Approach

Cited by 10 publications

References 18 publications

Surgical palliation for an infant with hypoplastic left heart syndrome and congenital lobar emphysema: a case report

Surgical palliation for an infant with hypoplastic left heart syndrome and congenital lobar emphysema: a case report

Case Report: 5 Cases of Variant Hypoplastic Left Heart Syndrome Diagnosed on Prenatal Fetal Ultrasound

The heart in congenital diaphragmatic hernia: Knowns, unknowns, and future priorities

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