Eckardt Trowitzsch, Martin Schneider, Andreas Urban, Boulos Asfour
Vestische Kinder- und Jugendklinik, University of Witten/Herdecke.
Germany
Clinical Research in Cardiology
Clin Res Cardiol 2006; 95: 338-343
DOI: 10.1007/s00392-006-0383-x
Abstract
In a newborn with anal atresia and urethral valves an incomplete VACTERL association, was diagnosed and a colon anus praeter was placed. Sweating and heart murmur led to cardiac diagnostics. By 2D colour Doppler echocardiography a huge atrial septal defect and pulmonary venous stenoses were diagnosed. Additionally, a pulmonary sling combined with an aortopulmonary window (AoPAw) was suspected and later confirmed by angiography. Heart failure developed and closure of the AoPAw, transplantation of the left pulmonary artery and closure of the atrial septal defect was performed at the age of 4 weeks. But the patient did not improve. Pulmonary hypertension with suprasystemic pressure in the right ventricle originating from a stenosis of the new orifice of the left pulmonary artery and obstruction of the right pulmonary veins was diagnosed by echocardiography and confirmed by angiography. At the age of 8 months the orifice of the left pulmonary artery was enlarged by a patch, the obstruction of left sided pulmonary veins was opened, and the rightsided pulmonary veins were newly implanted into the left atrium. Finally the inter-atrial communication was closed by a patch plastic. Again, postoperatively the patient improved only slightly. At the age of 10 months trans-septal catheterisation was performed. Angiography revealed a successful balloon dilatation of the long obstruction of the right pulmonary veins. Later on, an absorbable magnesium stent was implanted into the right upper pulmonary vein and medication with an endothelin antagonist was started. Temporarily the patient improved significantly. Within 6 weeks, right ventricular systolic pressure was again suprasystemic due to extreme inflow obstruction of the right pulmonary vein diagnosed by echocardiography. Severe heart failure developed and at the age of 1 year the patient died.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Right Heart Failure Associated with Stenosis or Atresia of Pulmonary Veins
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Catheter-mediated Interventions: Efficacy or Lack of Efficacy
Year of Publication: 2006
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No