An Uncommon Congenital Abnormality Discovered Using Multimodality Cardiac Imaging in an Elder Hospitalized For Decompensated Heart Failure

We report the case of a 77-year-old patient admitted for worsening dyspnea, palpitations and dizziness. The electrocardiogram (ECG) showed atrial fibrillation (AF) and left bundle branch block (LBBB). Clinical examination showed hypoxemia (SaO2 87% room air), jugular venous distension, pitting edema, arrhythmic heart sounds, a splitting of the second heart sound and systolic murmur (III-IV/VI) in the tricuspid area. Transthoracic echocardiography (TTE) showed right heart dilation, moderate tricuspid regurgitation, biventricular hypertrophy, and a dilated coronary sinus (CS). Agitated saline injection in the left basilar vein raised a suspicion of persistent left superior vena cava (PLSVC) to coronary sinus fistula. Transesophgaeal ecocardiography (TOE) showed a superior sinus venosus defect, with bidirectional shunt, and persistence of LSVC. CT angiography confirmed and detalied the malformation features. A low suspicion for congenital defects in older adults presents a significant diagnostic challenge. Multimodality cardiac imaging is critical in determining the etiology of right heart dilation, and knowledge of the anatomy and physiology of various shunt lesions is essential for clinicians.

The Vein Of Galen Aneurysmal Malformation Associated With Sinus Venosus Defect And Anomalous Pulmonary Veins

he Vein of Galen Aneurysmal Malformation (VGAM) is a rare cerebral arteriovenous shunt, which may be associated with a congenital cardiac defect. Embolisation of the VGAM is safer in infancy, even it may be undertaken in the neonatal period if necessary.

Abstract 14310: Elephant Sitting on My Chest: A Tale of Two Vessels

Introduction: A dilated main pulmonary artery (MPA) seen in congenital heart disease (CHD) or pulmonary arterial hypertension (PAH) is a rare cause of angina due to left main coronary artery (LMCA) compression. Case: A 49 year old male with no prior cardiac history presented with substernal chest pain. He reported exercise intolerance for several years. On exam he had an oxygen saturation of 85%, loud P2, fixed split second heart sound and ejection click. Electrocardiogram showed right ventricular (RV) hypertrophy and no significant ST changes. Laboratory data revealed a negative troponin. An enlarged main and right pulmonary artery (RPA) (Figure 1A) was seen on computed tomography (CT) chest angiography for pulmonary embolism (CTPE) and on echocardiography he had a severely dilated RV with decreased function (Figure 1B,1C). Cardiac catheterization showed a step up in saturations from 56 to 71%, Qp:Qs of 1.5 mean MPA pressure of 53 mmHg, pulmonary capillary wedge pressure of 11mmHg and pulmonary vascular resistance (PVR) of 7WU. A slit-like narrowing of LMCA ostium was seen on coronary angiography and intravascular ultrasound (Figure 1D). He was diagnosed with a sinus venosus defect, anomalous right upper pulmonary venous drainage and suspicion for extrinsic compression of LMCA by the dilated RPA that was confirmed on cardiac CT (Figure 1E-I). He was started on triple therapy for PAH with a significant reduction in PA pressures (mean 36 mmHg and PVR 2.1 WU). Percutaneous stenting of the LMCA was deferred and he underwent successful sinus venosus defect repair with a 5mm fenestrated patch, baffling of the anomalous pulmonary veins and a 32 mm interposition graft in the MPA with reduction plasty of MPA/RPA. Conclusion: This is an unusual presentation of unrepaired CHD with angina due to a compressed LMCA from a dilated pulmonary artery. Treatment of the underlying PAH as well as repair of the defect including reduction plasty of the MPA is a consideration for symptomatic relief.

Long-Term Outcomes of the Double-Barrel Technique for Superior Sinus Venosus Defect With Partially Anomalous Pulmonary Venous Connection

Background: Repair of superior sinus venosus defect with high partially anomalous pulmonary venous connection (PAPVC) using an intracardiac baffle may be complicated by systemic or pulmonary venous pathway obstruction and sinus nodal dysfunction (SND). Our surgical strategy for repair of all types of superior sinus venosus defect has evolved chiefly to avoid the abovementioned complications and preserving the growth potential of the superior cavoatrial junction. Methods: Between 2007 and 2019, fifty consecutive patients aged 2 to 60 (mean, 17.6±16.7) years underwent repair of superior sinus venosus defect using the double-barrel technique as described. The anomalous pulmonary veins drained into the superior cavoatrial junction in 17 patients and more than 2 cm above the cavoatrial junction in 33 patients. Results: There were no early or late deaths and no reoperations. At a mean follow-up of 103.9 (±50.2) months, all survived the operation, and actuarial freedom from SND was 97.9% (±standard error, 0.02%; 95% CI: 0.86-0.99). No patient had systemic or pulmonary venous pathway obstruction. A permanent pacemaker was required in one (2%) patient for sick sinus syndrome. Conclusions: The double-barrel method is an expedient, safe, and effective technique in superior sinus venosus defect. It provides dual drainage of superior vena cava preserving the superior cavoatrial junction without causing systemic or pulmonary venous pathway obstruction and can be utilized in all cases including those with high PAPVC. Preservation of the cavoatrial junction and use of autogenous atrial tissue for systemic venous pathway avoids SND and preserves growth potential.

Antiplatelet and Antithrombotic Therapy After Patent Foramen Oval and Atrial Septal Defect Closure

: Pathologies of the atrial septum include different interatrial communications varying from patent foramen ovale (PFO) to actual defects. Atrial septal defects (ASDs) may be localized within the fossa ovalis such as the secundum type ASD or outside the region of fossa ovalis, such as the ostium primum defect and sinus venosus defect. Over the last decades, the percutaneous closure of interatrial shunts has become a feasible and safe method. During these procedures, the delicate balance between thrombotic risk, device sealing process and bleeding risk is crucial. In this review, we sought to describe current available data on the antiplatelet and antithrombotic management of patients after percutaneous ASD or PFO closure.