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[Raghib's syndrome associated with cor triatriatum--a rare surgical case report]
Insights
This study details a rare surgical correction of Raghib's syndrome combined with cor triatriatum in a congenital heart disease patient. The successful procedure involved redirecting blood flow and repairing cardiac defects, preventing potential complications.
Area of Science:
- Cardiology
- Congenital Heart Disease Surgery
Background:
- A 27-year-old male with congenital heart disease underwent pre-operative evaluation for a cardiac malformation.
- Diagnostic imaging revealed an incomplete endocardial cushion defect and persistent left superior vena cava draining into the left atrium.
Observation:
- Intraoperative findings during surgical correction of Raghib's syndrome identified an additional rare condition: cor triatriatum.
- Raghib's syndrome involves abnormal left superior vena cava drainage, an atrial septal defect, and absent coronary sinus.
Findings:
- Surgical repair included redirecting left superior vena cava blood flow to the right atrium using a Dacron graft.
- Mitral valvoplasty, excision of an obstructing diaphragm, and atrial septation were successfully performed.
Implications:
- This case highlights the extreme rarity of surgically correcting Raghib's syndrome concurrently with cor triatriatum.
- The successful intervention aimed to prevent secondary complications like brain abscess and improve patient outcomes.
Abstract:
A 27-year-old male with a history of congenital heart disease was admitted for pre-operative evaluation of a cardiac malformation. Echocardiography and cardiac catheterization revealed an incomplete endocardial cushion defect with a persistent left superior vena cava which drained into the left atrium but echo-cardiographic evidence of an abnormal intra-atrial septum was not found. The patient was placed on cardiopulmonary bypass and prepared for the surgical correction of his primary cardiac lesion. Intra-cardiac examination during reconstruction of Raghib's syndrome also revealed the presence of cor triatriatum. Reghib's syndrome is characterized by the combination of abnormal drainage from the left superior vena cava into the left atrium, the presence of an atrial septal defect and the absence of a coronary sinus. To prevent secondary complications such as a brain abscess, we redirected blood flow from the left superior vena cava to the right atrium utilizing a trimmed woven dacron vascular graft that was placed in the intra-atrial position. Mitral valvoplasty, excision of the obstructing diaphragm and atrial septation were also performed successfully. Although the literature has described the surgical repair of Raghib's syndrome, its correction in combination with cor triatriatum is considered to be extremely rare.