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Double-horned or caplike right ventricle: diagnosis and operative treatment
A J Muster1, C Mavroudis, C L Backer
1Division of Cardiology, Children's Memorial Hospital, Chicago, Illinois, USA.
Insights
Double-horned right ventricle is a rare congenital heart defect with unusual morphology. Surgical correction is recommended for most patients with this condition.
Area of Science:
- Congenital heart disease
- Pediatric cardiology
- Cardiac surgery
Background:
- Double-horned right ventricle is a rare congenital cardiac malformation.
- This condition presents unique diagnostic and operative challenges.
Observation:
- A retrospective analysis of 3 new patients and 4 literature cases was performed.
- Cardiac catheterization, cineangiography, and echocardiography were used for diagnosis.
- Surgical interventions included palliative and corrective operations.
Findings:
- Distinct morphologic features on imaging differentiate double-horned right ventricle from other complex heart anomalies.
- The condition involves an unusual ventricular shape, possibly due to incomplete right ventricular development.
- It is consistently associated with a double right ventricular outlet.
Implications:
- Accurate diagnosis through advanced imaging is crucial.
- Anatomic surgical correction is the suggested approach for typical cases.
- Further research into the developmental origins and surgical outcomes is warranted.
Background:
Three patients reported here and 4 from the literature serve as background for the state-of-art diagnostic and operative considerations for an unusual congenital cardiac malformation: double-horned or caplike right ventricle.
Methods:
This is a retrospective analysis of cardiac catheterization, cineangiography, and two-dimensional echocardiography findings, as well as palliative and corrective operations in 3 previously unreported patients. Four patients from the literature are reviewed.
Results:
Characteristic morphologic features recognizable by invasive and noninvasive imaging distinguish double-horned right ventricle from complex malformations such as criss-cross hearts, superior-inferior ventricles, and univentricular hearts with a small outflow chamber.
Conclusion:
Double-horned or caplike right ventricle is a congenital malformation characterized by an unusual ventricular morphology, which may be the result of incomplete development of the right ventricle. The two-horned appearance may be secondary to an absence of the apical trabeculated compartment, with the left ventricle wedged between the two horns. It is invariably associated with double right ventricular outlet. Surgical experience so far suggest that most patients with typical double-horned right ventricle should be considered for anatomic surgical correction.