Hypoplastic Left Heart Syndrome

Steven, James M.; Marino, Bradley S.; Jobes, David R.

doi:10.1016/b978-032301281-2.50042-4

Cited by 4 publications

(7 citation statements)

References 104 publications

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“…Seldom anomalous pulmonary vein drainage directly to the right atrium or either vena cava is observed (11–14). The ductus arteriosus in HLHS is typically variable in size, but of normal anatomical location (12).…”

Section: Introductionmentioning

confidence: 99%

“…The LV in a child with HLHS is a non-functional anatomical structure (14). The systolic volume is ejected into the truncus pulmonalis, where the blood flow is divided.…”

Section: Introductionmentioning

confidence: 99%

“…The hypoplastic ascending aorta serves as a common coronary artery, thus a pre-ductal aortic coarctation may compromise an adequate perfusion of the hypertrophied right heart muscle (15). Since the blood flow to and from the left ventricle is minute or absent, the atrial septal defect provides the only significant exit from the left atrium for the pulmonary venous blood flow (14). Because of systemic and pulmonary venous blood flow merging in the right atrium, the approximate arterial blood oxygen saturation is about 80%.…”

Section: Introductionmentioning

confidence: 99%

“…In an attempt to maintain an adequate perfusion in the systemic circulation the cardiac output increases, however, with the consequent increase in cardiac effort, the compensatory mechanisms are short-lived. With rapid development of hypoxia and tissue ischemia metabolic acidosis ensues (1, 14, 15). A significant pulmonary blood flow limiting factor is the left atrial pressure, which is for the most part determined by the size of the atrial septal defect.…”

Section: Introductionmentioning

confidence: 99%

“…A significant pulmonary blood flow limiting factor is the left atrial pressure, which is for the most part determined by the size of the atrial septal defect. As a result, a moderately restrictive flow from the left to right atrium with retained adequate pulmonary venous drainage might be advantageous (14). …”

Section: Introductionmentioning

confidence: 99%

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Hypoplastic left heart syndrome: a review

Gobergs¹,

Salputra²,

Lubaua³

2016

AML

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Background.Hypoplastic left heart syndrome (HLHS) is an etiologically multifactorial congenital heart disease affecting one in 5,000 newborns. Thirty years ago there were no treatment options for this pathology and the natural course of the disease led to death, usually within the first weeks of life. Recently surgical palliative techniques have been developed allowing for a five-year survival in more than half the cases.Materials and methods.We reviewed literature available on HLHS, specifically its anatomy, embryology and pathophysiology, and treatment. The Pubmed and ClinicalKey databases were searched using the key words hypoplastic left heart syndrome, foetal aortic valvuloplasty, foetal septoplasty, Norwood procedure, bidirectional Glenn procedure, Fontan procedure, hybrid procedure. The relevant literature was reviewed and included in the article. We reported a case from Children’s Clinical University Hospital, Riga, to illustrate treatment tactics in Latvia.Results.There are three possible directions for therapy in newborns with HLHS: orthotopic heart transplantation, staged surgical palliation and palliative non-surgical treatment or comfort care. Another treatment mode – foetal therapy – has arisen. Staged palliation and full Fontan circulation is a temporary solution, however, the only means for survival until heart transplantation. Fifty to 70% of patients who have gone through all three stages of palliation live to the age of five years.Conclusions.The superior mode of treatment is not yet clear and the management must be based on each individual case, the experience of each clinic, as well as the financial aspects and will of the patient’s parents.

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Section: Introductionmentioning

confidence: 99%

“…The LV in a child with HLHS is a non-functional anatomical structure (14). The systolic volume is ejected into the truncus pulmonalis, where the blood flow is divided.…”

Section: Introductionmentioning

confidence: 99%