A female child, 5 years old complaining of dyspnea on exertion for about 1 year and episodes of cyanosis, Her weight was 10.5 Kg, height was 88 cm. She was not cyanotic at the time of examination. There was a harsh and loud holo systolic murmur allover the pericordium. Echocardiography is attached.
Multple VSDs. Signs of impending CHF. Cardiac surgical intervention should be considered.
"Swiss cheese" type of multiple Ventricular Septal Defects (VSDs).The surgical management should be offered for complete repair. I agree with Dr R. Rowley
Multiple VSDs, to fullfill criteria of swiss cheese VSDs the VSDs must be spreaded in the muscular septum, inlet and outlet septum. here it seems to be only in the muscular septal parts. This echo is indicating still recirculation. But before any heart surgery should be performed, please perform a catherization to measure the pulmonary resistance. If the pulmonary resistance is above 50% of the systemic circulation consider a pulmonary banding before corrective surgery, PA Banding should reduce the PA pressure at least by 50% of preassure before. Do a re cath after a year after banding and see if the pulmonary resistance is reduced. If the resistance is below 30% of the systemic do a VSD closure and debanding of PA.
The echo shows multiple mid apical and midmuscular VSDs which are shunting predominantly left to right. As the child is already 5 years old and there is severe PAH on the echo, a diagnostic cardiac catheterization study is worthwhile to check the PVRi and PVR/ SVR ratios and PAH reversibility with inhaled nitric oxide, Sildenafil and oxygen. If the PVR is reversible (< 3 wood units at baseline or post pulmonary vasodilators) the patient can undergo a surgical VSD closure. If the PVR is high and only partially reversible, she will require a fenestrated VSD closure and oral Sildenafil, Bosentan post surgery. Going by the findings of a loud systolic murmur and cardiomegaly on echo, chances are cath data will be conducive for a complete VSD closure.
Multiple muscular VSDs .From TR gradient it is obvious that there is severe pulmonary arterial hypertension.However ins spite of severe PAH,there is dilation of LV and RA,so threre may be possibility of hyperkinetic pulmonary arterial hypertension rather than permanant structural changes in pulmonary tree.PVR measurement is must before taking dicision of surgical closure.I fully agree with opinion of Dr supratim sen
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