Adults with congenital complete heart block: 25-year follow-up

Cardiac resynchronization therapy (CRT) is an accepted therapy for adults with dilated cardiomyopathy (DCM) and has been shown to improve ejection fraction and functional capacity. CRT has been used in children for over 4 years with a variety of indications which differ substantially from those commonly used for adult patients. We reviewed the results of CRT in the combined Stanford-UCSF pediatric electrophysiology program over the past 4 years, in order to evaluate efficacy of CRT in different clinical scenarios. We have provided CRT to 31 patients (15 female) between 2003 and 2007. The mean age at implantation was 7.3 ± 6.6 years. 21 patients had congenital heart disease (CHD) of whom 4 had single ventricle physiology. 5 patients had DCM and 5 had congenital complete heart block with reduced LV systolic function (CCHB). All patients with DCM met criteria for CRT in adults (QRS duration < 120 msec and EF > 35%). Implantation in the other patient groups was performed at the discretion of the physician. Mean follow-up was 26 ± 15 months. Ejection Fraction (EF), by echo, was 30 ± 11% at baseline which improved to 41 ± 19% at last follow-up (p < 0.05). EF as well as LV end diastolic volume and LV end systolic volume showed immediate improvement followed by additional continued improvement over the study period. CHD and CCHB patients reported improved symptoms and had improved cardiac indices by echo. DCM patients, however, showed no improvement and instead had a gradual decrease in ejection fraction over the study period (30 ± 11% to 18 ± 11%, p< 0.01). Two of the 5 DCM patients were transplanted, and one died of pump failure. Conclusions: CRT is efficacious in selected patients with congenital heart disease, or those with congenital complete heart block. The role of CRT in pediatric patients with dilated cardiomyopathy is less clear, but adult selection criteria may not be applicable to children. Comparison of EF preCRT and at last follow-up

Download Full-text

Noncompaction of Ventricular Myocardium in a Patient with Congenitally Corrected Transposition of the Great Arteries Treated Surgically: Case Report

The Heart Surgery Forum ◽

10.1532/hsf98.20041142 ◽

2005 ◽

Vol 8 (2) ◽

pp. 110 ◽

Cited By ~ 13

Author(s):

Riza Dogan ◽

Omer Faruk Dogan ◽

Mehmet Oc ◽

Umit Duman ◽

Suheyla Ozkutlu ◽

...

Keyword(s):

Case Report ◽

Heart Block ◽

Complete Heart Block ◽

Ventricular Myocardium ◽

Situs Inversus Totalis ◽

Noncompaction Of Ventricular Myocardium ◽

Congenital Complete Heart Block ◽

Congenitally Corrected Transposition ◽

Corrected Transposition

Noncompaction of the ventricular myocardium is a rare disorder that represents numerous prominent trabeculations and intratrabecular recesses in the ventricles. It is believed to represent not only an arrest in endomyocardial morphogenesis but also an unclassified cardiomyopathy. The pathology has been almost invariably associated with other congenital cardiac malformations. A female patient with noncompaction of the myocardium of both ventricles and congenitally corrected transposition of the great arteries (cTGA), situs inver-sus totalis, and atrial and ventricular septal defects is described. When she was 7 days old a permanent pacemaker was implanted because of complete heart block. Prazosin (Minipress), an a -receptor blocker, was administered, and the cardiac ejection fraction showed a striking increase from 20% to 42%. Despite careful and regular follow-up evaluations, the general condition of the patient slowly worsened. Five months after surgery she died of hepatorenal failure and low cardiac output. This case report is thought to be the first description of congenital complete heart block, cTGA, and situs inversus totalis with noncompaction of the myocardium of both ventricles.

Download Full-text