The Hidden Dangers of Additional Shunts in Patent Foramen Ovale Treatment

Abstract

Background: Unroofed coronary sinus (UCS) is a rare congenital defect that causes a left-to-right shunt, often associated with anomalies such as persistent left superior vena cava. Its diagnosis and management are challenging.

Case summary: A 49-year-old woman with worsening dyspnea, orthopnea, and peripheral edema unresponsive to diuretics was referred for evaluation. She had a history of epilepsy and had had a patent foramen ovale (PFO) closure 1 year before. Transthoracic echocardiography revealed right ventricular dilation, preserved biventricular function, and increased pulmonary pressure. Imaging and right heart catheterization confirmed UCS and partial anomalous pulmonary venous connection (PAPVC). The coronary sinus roof was reconstructed, and a modified Warden procedure corrected the PAPVC.

Discussion: This case highlights the rare coexistence of PFO, UCS, and dual PAPVC, emphasizing the need for comprehensive diagnostic evaluation and tailored surgical intervention for complex congenital defects.

Keywords: anomalous pulmonary venous connection; atrial septal defect; congenital heart defect; patent foramen ovale; unroofed coronary sinus.

Graphical abstract

Figure 1

Electrocardiogram at Admission Sinus rhythm with normal atrioventricular conduction and incomplete right bundle branch block.

Figure 2

Transthoracic Echocardiography (A) An apical 4-chamber view depicting the patent foramen ovale occluder placed into the interatrial septum (blue arrow). (B) An apical 4-chamber view revealing a color Doppler shunt between the left and the right atrium. (C) Parasternal long axis view and (D) apical 2-chamber view of dilated coronary sinus. In the latter, color Doppler revealed an apparent jet from the coronary sinus through the left atrium.

Figure 3

Right Heart Catheterization Right heart catheterization. (A) Coronary sinus opacity after contrast injection into the left pulmonary artery. (B) Superior vena cava opacity after contrast injection into the right pulmonary artery.

Figure 4

Cardiac Anatomy at Computed Tomography Angiography (A to C) Cardiac computed tomography angiography (CTA) showing unroofed dilated coronary sinus (red arrow in B and C) with anomalous communication with left atrium and contiguous left pulmonary veins (blue arrow in A to C). (D) Cardiac CTA venous phase showing partial anomalous venous return of 3 right pulmonary veins draining upper and middle lobe into superior vena cava (asterisk in D) 3 cm cranially from the cavoatrial junction.

Figure 5

Three-Dimensional Reconstruction of the Coronary Sinus White arrows indicate the coronary sinus. The right atrium is represented in yellow, and the left atrium and pulmonary veins are in purple.

Figure 6

Intraprocedural Pictures of the Congenital Defect (A) Left atrium after interatrial septum and occluder removal. The white arrow indicates the orifice of the unroofed coronary sinus. The transparent catheter (red arrow) was inserted from the natural orifice of the coronary sinus in the right atrium, crossed the coronary sinus, and emerged from the unroofed portion of the coronary sinus in left atrium. The blue arrow shows the orifice of the inferior pulmonary vein. (B) Anatomy of the anomalous pulmonary venous return (asterisks) draining in the SVC. (C) Redirection of pulmonary venous return into the left atrium. The blue arrow indicates the pericardial encircling the ostium of the SVC, and the white arrow shows the pericardial patch used to close the proximal stump of the SVC. (D) Systemic venous drainage reestablishment through the vascular prosthesis (asterisk). Ao = aorta; SVC = superior vena cava.

Figure 7

Postoperative Cardiac Evaluation at Computed Tomography Angiography (A and B) Postoperative cardiac computed tomography angiography showing reconstructed coronary sinus with pericardial patch (red arrow in A and B), without anomalous communication with left atrium and contiguous left pulmonary veins (blue arrow in A and B). (C and D) Right partial anomalous venous return exclusion and drainage in left atrium (yellow arrow) with superior vena cava reconstruction (asterisk).

Figure 8

Anatomical Representation of the Congenital Defect Before and After Surgical Correction (Left) Congenital defect before surgical correction. The defects included a partial anomalous pulmonary venous connection (PAPVC) draining into the superior vena cava (SVC), the unroofed coronary sinus, and the patent foramen ovale occluder. (Right) Corrected congenital defect. The occluder was removed, UCS was closed with a pericardial patch, and the SVC was transected distal to the PAPVC. The proximal stump of the SVC (now receiving only pulmonary venous flow) was redirected to the left atrium with a pericardial patch. The distal stump of the SVC (receiving systemic venous flow) was directed to the right atrium through a graft.

Visual Summary

Unroofed Coronary Sinus: Diagnosis and Treatment (A) Parasternal long-axis view upon presentation, revealing a dilated coronary sinus and right ventricle. (B) Angiography of the left pulmonary is followed by the opacification of a dilated coronary sinus due to the presence of an unroofed coronary sinus. (C) Angiography of the right pulmonary artery and concomitant opacification of the superior vena cava (SVC) and right atrium due to the presence of a partial abnormal pulmonary venous connections (PAPVC). (D) Preoperative CT scan, the red arrow points to the unroofed coronary sinus; the blue arrow shows the inferior pulmonary vein draining just above the opening of the unroofed portion of the coronary sinus. (E) Intraoperative identification of the PAPVC draining in the SVC (asterisks). (F) Blue arrow indicates the pericardial patch used to redirect pulmonary venous flow from the SVC after the unroofed coronary sinus defect was corrected. The white arrow points to the pericardial patch used to isolate the proximal stump the transected SVC.