Hypoplastic left heart

The hypoplastic left heart is a critical congenital malformation affecting newborns when the left ventricle (LV) is unable to provide cardiac output. It is a group of heart defects in which the structures of the left heart are not fully developed. The left ventricle is small and non-functional, the right ventricle provides both pulmonary and systemic circulation. The defect is usually associated with mitral or aortic valve atresia and aortic coarctation. The child survives the first days only due to the persistence of the arterial duct, the atrial septal defect or the persistent foramen ovale.

Manifestation
After closing the Botalla's duct, the disease manifests itself. Symptoms of heart failure (tachypnoea, low heart attack) usually occur within 2-4 days cardiac output, tachypnea, central cyanosis) and a shock (metabolic acidosis, peripheral perfusion disorder, anuria) gradually develops.

Diagnosis
Echocardiography.

Treatment
Prostaglandin E1 will allow the drug to remain open, and we indicate a surgical solution. Palliative is a multi-stage operation according to Norwood (single-chamber circulation can occur), the final solution is a heart transplant.

In addition, if right ventricular dysfunction (significant tricuspid regurgitation or aortic hypoplasia) is present, the defect is inoperable.

Termination of pregnancy is considered for prenatal detection of this defect.

Related Articles

 * Congenital heart malformations
 * acquired heart defects