Partial atrioventricular canal defect with cor triatriatum sinister: report of three cases

Abstract

or triatriatum is an uncommon but surgically correctable cause of pulmonary venous hypertension and congestive cardiac failure, with a reported incidence of 0.1% among children with congenital heart diseases. Association with partial atrioventricular canal defect (PAVCD) is even rarer, with only anecdotal reports appearing in the literature. In the classic form, cor triatriatum is characterized by the presence of a fibromuscular diaphragm that subdivides the left atrium into a proximal accessory chamber and a distal true chamber. Clinical SummariesPATIENT 1. A 14-year-old boy was seen with class 2 exertional dyspnea. Clinical examination revealed fixed splitting of the second heart sound in the pulmonary area and short systolic murmur (grade 3/6) at the apex. Chest radiography showed right atrial enlargement and plethoric lung fields. Transthoracic echocardiography (TTE) demonstrated the presence of PAVCD with moderate left atrioventricular valve regurgitation and a dilated coronary sinus, suggestive of left superior vena cava. PAVCD was repaired under cardiopulmonary bypass. Postoperative TTE showed turbulence in the supramitral area with suspected membrane positioned obliquely and superiorly within the left atrium. Angiocardiography demonstrated an hourglass-shaped left atrium because of cor triatriatum (Figure 1, A and B). The pulmonary artery wedge pressure was elevated (16-22 mm Hg, mean 16 mm Hg), with a gradient of 11 mm Hg from the left ventricular end-diastolic pressure.PATIENT 2. A 4-year-old girl was evaluated for recurrent respiratory tract infection. TTE revealed PAVCD with mild left atrioventricular valve regurgitation and a patent ductus arteriosus. During surgery, a diaphragm separating the left atrium in to a proximal pulmonary venous chamber and a distal true chamber with a 7-mm central communication was identified. The left atrial appendage was arising from the distal chamber. The membrane was excised, and the PAVCD was repaired successfully.PATIENT 3. An 18-year-old woman had PAVCD with moderate left atrioventricular valve regurgitation diagnosed by TTE. During the operation, while the surgeon was checking for the orifices of pulmonary veins, a thin diaphragm partitioning the atrium with a central hole of 12 mm was found. The coronary sinus was unroofed. The proximal chamber was communicating to right atrium by a stretched foramen ovale. The appendage was arising from the distal true chamber. The membrane was excised, and the rest of the repair was carried out uneventfully. DiscussionThese cases illustrate the importance of looking for associated lesions while managing congenital cardiac defects. The clinical picture of cor triatriatum depends on the size of the communication in the obstructing membrane 1 and the associated intracardiac defects. Fea-From the Departments of Cardiology and

Horseshoe Lung With Cor Triatriatum and Partial Atrioventricular Canal Defect

“…This PAH is generally reversible after appropriate surgical management with good long-term outcome. 1,5 Presentation of patients with cor triatriatum and AVCD is usually delayed, and age of presentation depends upon the size of communication between the common chamber and the left atrium. 5 Size of communication >7 mm leads to delayed development of PAH while size of communication <3 mm causes early development of pulmonary venous and arterial hypertension.…”

Cor Triatriatum Sinister: A Rare Cause of Dyspnea on Exertion in Adulthood: Case Report

“…2 Semptomlar ile zar üzerindeki fenestre alanın bü-yüklüğü arasında ilişki vardır. 4 Özellikle 3 mm'nin altındaki fenestre alanın şiddetli semptomlara yol açtığı rapor edilmiştir. 4 Hastalık genellikle tek başına görülmesine karşın, atriyal septal defekt, patent foramen ovale, parsiyel venöz dönüş anomalisi gibi diğer doğumsal kalp hastalıkları eşlik edebilir.…”

“…4 Özellikle 3 mm'nin altındaki fenestre alanın şiddetli semptomlara yol açtığı rapor edilmiştir. 4 Hastalık genellikle tek başına görülmesine karşın, atriyal septal defekt, patent foramen ovale, parsiyel venöz dönüş anomalisi gibi diğer doğumsal kalp hastalıkları eşlik edebilir. 1 Hastalık sıklıkla yaşamın erken yılarında tanınmasına karşın, nadiren erişkin yaşamda da karşımıza çıkabilmektedir.…”

Cor Triatriatum Sinistrum: Surgical Experience in Taiwan

“…To our knowledge, only 5 cases of complete atrioventricular canal defect and 11 cases of partial defect have ever been reported. [18][19][20][21][22] Preoperative echocardiography failed to identify the CT but angiocardiography subsequently confirmed the anomaly. We used a two-patch technique to successfully repair the atrioventricular canal defect in this patient, who is only the fourth surgical survival of a complete atrioventricular canal defect according to our literature search.…”