Isolated Congenital Unilateral Absence of the Right Pulmonary Artery and Left Atrial Flutter: Are they Related?
This is a 51-year-old patient referred for episodes of atypical atrial flutter, with positive P waves in the inferior and precordial leads and negative in leads I and aVL (Fig. 1) at 290 ms cycle length. The arrhythmia recurred in spite of several sequential attempts with antiarrhythmic drugs (quinidine, propafenone, flecainide and amiodarone). The patient had episodes of hemoptisis during childhood, but no instrumental diagnostic procedure was performed at that time. While on sinus rhythm, the patient did not complain of any symptom and was in NYHA class I. On admission, chest X-ray (Fig. 2) showed rightward displacement of the heart with an asymmetric chest wall. Echocardiography showed mild enlargement of both atria with minimal mitral regurgitation. A 16-slice computed tomography showed a poorly expanded and dystrophic right lung with evidence of several bollous cavities (Fig. 3a). Volume-rendering reconstruction clearly evidenced the absence of the right pulmonary artery (Fig. 3b). Reconstruction of the circulation in the right lung of this patient has been reported in detail elsewhere . Briefly, the main collateral vessels to right lung were present, arising from a dilated right internal mammary and from the celiac trunk, but a ventilation-perfusion scintigraphy showed the absence of perfusion with almost normal ventilation in the right lung. Normal ventilation-perfusion was observed in the left lung. Three-dimensional reconstruction of the left atrium and pulmonary veins (Fig. 4) showed enlarged ostia of the left pulmonary veins, while the right pulmonary veins were of reduced diameter and had only minimal branching. The patient was admitted to the hospital during persistent recurrence of atrial flutter.
KeywordsPulmonary Vein Left Atrium Atrial Flutter Left Pulmonary Vein Left Superior Pulmonary Vein
Unable to display preview. Download preview PDF.