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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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Stenosis and atresia of the mitral valve, the hypoplastic left heart syndrome
Introduction

Mitral stenosis: the annulus is narrow, hypoplastic. The leaflets are thickend or fused with short chordae.

Mitral atresia:> the valve is completely absent with a dimple in the usual site of the valve, less common there is an imperforate membrane.

Stenosis or atresia of the mitral valve is the part of the hypoplastic left heart syndrome.

Hypoplastic left heart syndrome is a critical congenital heart defect with a small left ventricle and stenosis or atresia of the aortic and the mitral valve.

Macroscopic appearance
  • there is a wide spectrum of changes of the mitral valve, left ventricle and aorta
  • the combinations of the mitral stenosis and aortic atresia and combination of mitral and aortic stenosis are the most frequent
  • the whole aortic arch is strongly hypoplastic (the aorta is filiform)
  • the coronary arteries are supplied by reverse flow from the patent ductus arteriosus; the pulmonary artery is dilated
  • the left ventricle is always hypoplastic. With mitral atreis the left ventricle is extremely hypoplastic with thick walls and slitlike cavity or no cavity is present
  • with mitral stenosis a cavity in the small left ventricle is developed, the endocardium is fibrously thickened (fibroelastosis)
  • the right ventricle is hypertrophic pumping all the blood flow
Clinical signs
  • this critical defect manifests in the first days of life as the ductus closes by severe heart failure or shock
  • the affected infants uniformly die within few days without urgnet surgery
  • the defect is corrected in several steps (Norwood surgery)
  • after reconstruction the right ventricle works as the systemic ventricle but it cannot fully compensate the the normal left ventricle (right ventricle is a low-pressure pump)
  • in some cases this defect is inoperabile or could be corrected only by neonatal heart transplantation
  • individuals with hypoplastic left heart usually do not have any other congenital defects; hypoplastic left heart syndrome is not a typical part of other syndromes
Classification

Hypoplastic left heart syndrome is associated with premature (fetal) closure of foramen ovale in approximately 10% of the cases. The presence of intact atrial septum or highly restrictive (small) foramen ovale is a predictor of poor outcome in infants with hypoplastic left heart. Premature closure of foramen ovale is associated with coarctation of the aorta, an isolated form without any heart malformation is quite rare.

Pictures

Hypoplastic left heart syndrome, fibroelastosis of the left ventricle endocardium, fetus: Hypoplasia of the left ventricle, fibroelastosis, Macro, autopsy (72063)

Hypoplastic left heart with fetal closure of the foramen ovale: Hypoplastic left heart syndrome, Macro, autopsy (73189) Hypoplastic left heart syndrome, Macro, autopsy (73190) Hypoplastic left heart syndrome, Macro, autopsy (73191) Hypoplastic left heart syndrome, Macro, autopsy (73192) Hypoplastic left heart syndrome, Macro, autopsy (73193)

Another case of hypoplastic left heart with fetal closure of the foramen ovale: Hypoplastic left heart syndrome, Macro, autopsy (73194) Hypoplastic left heart syndrome, Macro, autopsy (73195) Hypoplastic left heart syndrome, Macro, autopsy (73196) Hypoplastic left heart syndrome, Macro, autopsy (73197) Hypoplastic left heart syndrome, Macro, autopsy (73198) Hypoplastic left heart syndrome, Macro, autopsy (73199)

Hypoplastic left heart: Hypoplastic left heart syndrome, Macro, autopsy (73292)

Hypoplastic left heart syndrome, ultrasound video: Hypoplastic left heart syndrome, Macro, autopsy (73664) Hypoplastic left heart syndrome, 1st trimester, Ultrasound, video (74011)

Hypoplastic left heart syndrome: Hypoplastic left heart syndrome, Macro, autopsy (73665) Hypoplastic left heart syndrome, Macro, autopsy (73666) Hypoplastic left heart syndrome, Macro, autopsy (73667) Hypoplastic left heart syndrome, Macro, autopsy (73668) Hypoplastic left heart syndrome, Macro, autopsy (73669) Hypoplastic left heart syndrome, Macro, autopsy (73670) Hypoplastic left heart syndrome, Macro, autopsy (73671) Hypoplastic left heart syndrome, Macro, autopsy (73672)

Case study
Hypoplastic left heart syndrome 1
Marta Ježová
History

24-week fetus, mitral atresia, aortic atresia, hypoplastic left heart syndrome.

Pictures

Hypoplastic left heart syndrome, base of the heart: Hypoplastic left ventricle, Macro, autopsy (72065)

Hypoplastic left heart syndrome, atresia of the mitral valve, left ventricle: Hypoplastic left ventricle, mitral atresia, Macro, autopsy (72062)

Hypoplastic left heart syndrome, hypertrophy of the right ventricle: Right ventricle in left heart hypoplasia, Macro, autopsy (72064)

Hypoplastic left heart syndrome, slit-like left ventricle at mitral atresia: Hypoplastic left heart, Macro, autopsy (72144)

Case study
Hypoplastic left heart syndrome 2
Marta Ježová
History

Immature 31-week newborn from twin pregnancy, mitral stenosis, aortic stenosis, hypoplastic left heart syndrome.

Pictures

Hypoplastic left heart syndrome: Hypoplastic left heart syndrome, Macro, autopsy (72371) Hypoplastic left heart syndrome, Macro, autopsy (72373)

Hypertrophy of the right ventricle: Hypoplastic left heart syndrome, Macro, autopsy (72375) Hypoplastic left heart syndrome, Macro, autopsy (72376)

Fibroelastosis of the left ventricle: Hypoplastic left heart syndrome, Macro, autopsy (72377)

Aortic valvular stenosis: Hypoplastic left heart syndrome, Macro, autopsy (72380)

Mitral stenosis: Hypoplastic left heart syndrome, Macro, autopsy (72372)