Noncompaction cardiomyopathy: a new mechanism for mitral regurgitation with distinct clinical, echocardiographic features and pathological correlations

Noncompaction cardiomyopathy [NCCM] is a primary, genetic cardiomyopathy with variable clinical manifestations that include mitral regurgitation [MR]. This study comprised patients diagnosed with NCCM and MR in two cardiac centers [King Abdul-Aziz Cardiac Center, Riyadh, Kingdom of Saudi Arabia and Sudan Heart Institute, Khartoum, Sudan], and seen in the period between 2002 and 2013. The study describes follow up, clinical, echocardiographic, and histopathological findings. Nineteen cases [85% females] were identified. Ten percent of the cases had relapses and remissions of heart failure. Echocardiographic features included leaflet retraction in all patients, characteristic malcoaptation, and a zigzag deformity of anterior leaflet in 57% of patients. Ruptured chordae were found in 15% of the patients. One patient had pathological examination of the mitral valve which showed myxomatous degeneration, and sclerotic and calcific changes. We describe and discuss a new mechanism for MR caused by NCCM with identifiable clinical and echocardiographic features, and pathological correlations

Outcome of low body weight [<2.2 kg] infants undergoing cardiac surgery

Introduction: Infants with low body weight [LBW] following cardiac surgery are a major challenge for the post cardiac surgery care unit. It has been observed that post surgery outcome for LBW infants is worse compared to the outcome of normal body weight infants

A study was conducted to compare post operative course and outcome of infants with body weight of 2.2 kg or less against infants with normal body weight who underwent similar cardiac surgeries

Methods: A retrospective review was performed for all infants below 2.2 kg who underwent cardiac operations at King Abdulaziz Cardiac Center from January 2001 to October 2011. Cases with LBW [Group A] were compared with matching group [Group B] of normal body weight infants who had similar cardiac surgeries and matching surgical risk category. The demographic, ICU parameters, complications, and short-term outcome of both groups were analyzed

Results: Two groups were formed, with 37 patients in Group A, and 39 patients in Group B. Except for weight [2.13 +/- 0.08 kg in Group A vs 3.17 +/- 0.2 kg in Group B], there was no statistical difference in demographic data between both groups. Cardiac procedures included coarctation repair, arterial switch, ventricular septal defect [VSD] repair, tetralogy of Fallot repair, systemic to pulmonary shunt and Norwood procedures. Patients in Group A had statistically significant difference from Group B in terms of bypass time [p = 0.01], duration of inotropes [p = 0.01], duration of mechanical ventilation [p = 0.004], number of re-intubations [p = 0.015], PCICU length of stay [p = 0.007] and hospital mortality: 13.5% in Group A vs 0% in Group B [p value 0.02]

Conclusion: Patients with LBW [<2.2 kg] underwent cardiac surgery with overall satisfactory results, but with increased risk of ICU morbidity and mortality

Dextrocardia, aortopulmonary window with transposition of the great arteries, case report

Transposition of the great arteries [D-TGA] in combination with aortopulmonary window [APW] is a very rare entity and carries high morbidity and mortality. Only few cases have been reported with this association. We report the first case of D-TGA and APW with mirror image dex-trocardia which was repaired successfully