Ann Thorac Surg 2009;87:964. doi:10.1016/j.athoracsur.2008.07.015
© 2009 The Society of Thoracic Surgeons
Images in Cardiothoracic Surgery
Diagnosis of Incorrectly Repaired Anomalous Right Pulmonary Veins in an Adult 40 Years After Primary Repair
Edward B. Savage, MDa,*,
George M. Kichura, MDa,
Mark D. Wittry, MDb,
Andrew C. Fiore, MDd,
Burt I. Bromberg, MDc,
Luke A. Bruns, MDc,
Dharam P. Goel, MDc,
Nicolin Neal, BAa
a Department of Heart and Vascular Services, St. John's Mercy Medical Center, St. Louis, Missouri
b Department of Radiology, St. John's Mercy Medical Center, St. Louis, Missouri
c Department of Pediatrics, St. John's Mercy Medical Center, St. Louis, Missouri
d Department of Surgery, SSM Cardinal Glennon Children's Medical Center, St. Louis, Missouri
* Address correspondence to Dr Savage, St. John's Mercy Heart Hospital, 625 S New Ballas Rd, Suite R7040, St. Louis, MO 63141 (Email: ebsavage99-3{at}yahoo.com).
In the 1960s, heroic repairs of the heart were accomplished. However, there was not a noninvasive method to confirm the correct repair. A young girl was diagnosed with a primum atrial septal defect. Repair was performed through a right thoracotomy, baffling the right pulmonary venous return through an absent atrial septum. At age 46, she presented with atrial fibrillation. The transesophageal echocardiogram demonstrated an enlarged right atrium and coronary sinus, and a small left atrium with normal valves. A 64-slice cardiac computed tomographic angiogram demonstrated the patch separating the right atrium from the pulmonary veins, with blood flow channeled into an enlarged coronary sinus in continuity with a persistent left superior vena cava (Fig 1A, coronal view; Fig 1B, sagittal view; and Fig 1C, three-dimensional reconstruction; asd = atrial septal defect; cta = computed tomographic angiography; ct = computed tomography; R = right; svc = superior vena cava). An accompanying video of the rotational view of three-dimensional reconstruction can be viewed on the Internet at: http://ats.ctsnetjournals.org/content/vol87/issue3/images/data/964/DC1/savage.avi.
A large bridging collateral was noted below the level of the kidneys. She had a left-to-right shunt of 1.8:1, mild pulmonary hypertension, and right atrial and ventricular enlargement. At reoperation, the right atrium was opened, demonstrating no coronary sinus opening, just those for the superior and inferior vena cava. A calcified dacron patch was removed. Evaluation demonstrated an intact atrial septum with a small patent foramen ovale and large coronary sinus ostium, previously mistaken for an absent septum. The fossa ovale was excised and a new patch was placed to redirect the right pulmonary venous return into the left atrium.
This case represents an interesting delayed presentation of anomalous right pulmonary veins misdiagnosed and incorrectly repaired as a child in the 1960s. The first repair channeled the right pulmonary blood directly through the right atrium to the systemic circulation. The high resolution computed tomographic images were key to understanding the incorrect repair and planning corrective surgery.
