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 [1]. 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.


Pulmonary Vein Left Atrium Atrial Flutter Left Pulmonary Vein Left Superior Pulmonary Vein 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Del Greco M, Centonze M, Marini M et al. Three-dimensional reconstruction of the right lung circulation in a patient with isolated absence of the right pulmonary artery. Eur Heart J 2006; 27: 1281.PubMedCrossRefGoogle Scholar
  2. 2.
    Pool PE, Vogel JHK, Blount SG. Congenital unilateral absence of a pulmonary artery. Am J Cardiol 1962; 10: 706–732.PubMedCrossRefGoogle Scholar
  3. 3.
    Bouros D, Pare P, Panagou P et al. The varied manifestation of pulmonary artery agenesis in adulthood. Chest 1995; 108: 670–676.PubMedGoogle Scholar
  4. 4.
    Harkel ADJT, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 2002; 122: 1471–1477.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 2008

Personalised recommendations