BOX SHAPED HEART
Septal and posterior leaflets of the tricuspid valve are long and redundant -and there proximal portion is plastered to the wall of RV particularly along septal wall - result in tricuspid regurgitation
- Proximal portion of RV cavity is “atrialized” but it contracts synchronously with the ventricle
- RV performance markedly reduced
patent fortamen ovale or atrial septal defect frequently seen - results in Rt-to-lt shunt inducing central cyanosis
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