• Lou Hofmeyr, Peter Pohlner, and Dorothy J Radford.
• Adult Congenital Heart Disease Unit, The Prince Charles Hospital, Brisbane, Queensland, Australia.
• Congenit Heart Dis. 2013 Nov 1;8(6):541-9.

BackgroundMultiple muscular ventricular septal defects (VSDs) in children can be difficult to treat and a range of techniques has been advocated. These include pulmonary artery banding, interventional catheter closure, and a variety of surgical approaches. When there are apical muscular defects and associated coarse trabeculations in the right ventricle (RV) producing a "Swiss cheese" pattern, a large patch extending on to the RV free wall and excluding part of the apex has been used.MethodsWe assessed four adult patients who had surgery 22 to 45 years ago to treat muscular VSD by patches which excluded the RV apex.ResultsAges ranged from 22 to 50 years. Re-presentations were for polycythemia, cyanosis, syncope, and atrial flutter. Echocardiography showed bidirectional flow from left ventricle to apex of RV, no pulmonary hypertension, small-sized RV with diastolic dysfunction, enlarged right atria, reopening of patent foramen ovale (PFO) in three, and positive bubble studies with right to left shunting in two. Catheterization confirmed elevated right atrial and RV end diastolic pressures. Two patients had evidence of hepatic cirrhosis. One woman had device closure of PFO, but has right heart failure. One man had redo surgical closure of VSD and PFO. Another patient is being considered for a Glenn shunt to take some load off RV.ConclusionsSurgical closure of muscular VSD by large patch with RV apical exclusion gives good early results. However, long term in adult life, the reduced size of RV, and diastolic dysfunction cause problems. These include reopening of PFO with cyanosis, right heart failure, cirrhosis, and arrhythmias.© 2013 Wiley Periodicals, Inc.

28 keywords...

hide…