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Sinus venosus atrial septal defect with partial anomalous pulmonary and hepatic venous drainage: a case report
Mahmood Hosseinzadeh Maleki1, Ali Heidari Bakavoli2, Hassan Birjandi3
1Department of Cardiac Surgery, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, Iran.
Insights
This case report details a rare congenital heart defect (CHD) involving sinus venosus atrial septal defect with multiple anomalies. Advanced imaging and surgical vigilance are crucial for managing such complex pediatric cardiac conditions.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Sinus venosus atrial septal defect (SVASD) is a rare congenital heart anomaly.
- Often associated with partial anomalous pulmonary venous drainage (PAPVD).
- Coexistence with anomalous hepatic venous drainage, cor triatriatum, and dual arterial ducts is exceptionally rare.
Purpose of the Study:
- To report a rare case of complex congenital heart disease.
- To highlight diagnostic and surgical challenges.
- To emphasize the importance of advanced imaging and intraoperative vigilance.
Main Methods:
- CT angiography and cardiac catheterization performed on an infant.
- Intraoperative findings revealed unexpected anomalies.
- Surgical management included ASD closure, PV rerouting, and ductal ligation.
Main Results:
- Diagnosis of SVASD with PAPVD and anomalous hepatic venous drainage.
- Unexpected intraoperative findings of dual arterial ducts and cor triatriatum.
- Successful surgical correction with an uneventful postoperative recovery.
Conclusions:
- This case underscores the rarity of this specific constellation of cardiac anomalies.
- Advanced imaging techniques are vital for preoperative diagnosis.
- Intraoperative vigilance is essential for managing unanticipated findings in complex CHD.
Background:
Sinus venosus atrial septal defect is an uncommon congenital cardiac anomaly frequently associated with partial anomalous pulmonary venous drainage. The coexistence of this defect with anomalous hepatic venous drainage, cor triatriatum, and two arterial ductal connections is extremely rare and may create diagnostic and surgical challenges.
Case Summary:
An eleven-month-old female infant with tachypnoea and growth retardation was found to have sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage and anomalous hepatic venous drainage to the coronary sinus on CT angiography. Cardiac catheterisation demonstrated a moderate left-to-right shunt and mildly elevated pulmonary artery pressure. Intraoperative assessment unexpectedly revealed two arterial ductal connections and cor triatriatum. Surgical management consisted of closure of the atrial septal defect, rerouting of pulmonary veins, and ligation of both arterial ductal connections. Hepatic venous drainage was left unaltered due to low flow and lack of hemodynamic effect. Postoperative recovery was uneventful.
Conclusion:
This rare constellation of anomalies highlights the importance of advanced imaging, careful preoperative planning, and intraoperative vigilance for unanticipated findings in CHD.
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