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

2005 ◽  
Vol 8 (2) ◽  
pp. 110 ◽  
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.

Abstract 2974: Midterm Results Of Resynchronization Therapy In Pediatrics: Do We Need New Guidelines?

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Anne M Dubin ◽  
Kathryn K Collins ◽  
George F Van Hare ◽  
V. M Reddy ◽  
Debra Hanisch ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Ejection Fraction ◽  
Dilated Cardiomyopathy ◽  
Heart Block ◽  
Congenital Heart ◽  
Complete Heart Block ◽  
Resynchronization Therapy ◽  
Congenital Complete Heart Block

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

Transient complete heart block following balloon pulmonary valvoplasty in a case of congenitally corrected transposition

1994 ◽  
Vol 4 (3) ◽  
pp. 301-303
Author(s):  
Leopoldo Romero ◽  
Ulrich Sigwart ◽  
Jane Somerville
Keyword(s):  
Heart Block ◽  
Septal Defect ◽  
Pulmonary Valve ◽  
Complete Heart Block ◽  
Pulmonary Arteries ◽  
Native Valve ◽  
Surgical Closure ◽  
The Right ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

AbstractBalloon pulmonary valvoplasty was performed in a patient with congenitally corrected transposition, aged 28 years, who had already undergone surgical closure of a ventricular septal defect and implantation of a conduit between the pulmonary arteries and the right-sided morphologically left ventricle. The patient developed stenosis of the conduit 22 years after the procedure as confirmed by cardiac catheterization and angiocardiography, which also showed stenosis of the native pulmonary valve. A balloon valvoplasty of the native valve was carried out with improvement of the gradient from the ventricle to the pulmonary arteries. The patient developed complete heart block immediately after the procedure, which reverted to first degree block in one week and to the previous cardiac rhythm within 21 days.

scholarly journals Maternal congenital complete heart block in pregnancy: a rare case report

2020 ◽  
Vol 9 (8) ◽  
pp. 3502
Author(s):  
Reena Sood ◽  
Harmandeep Kaur ◽  
Gaurav Mohan ◽  
Madhu Nagpal
Keyword(s):  
Case Report ◽  
Heart Block ◽  
Complete Heart Block ◽  
Early Recognition ◽  
Fetal Outcome ◽  
Team Work ◽  
Structural Defects ◽  
Satisfactory Condition ◽  
Escape Rhythm ◽  
Congenital Complete Heart Block

Complete heart block comprises complete absence of AV conduction - none of the supraventricular impulses are conducted to the ventricles. Perfusing rhythm is maintained by a junctional or ventricular escape rhythm. Typically, the patient will have severe bradycardia with independent atrial and ventricular rates. The incidence is 1 in 15,000 to 20,000 live births. Authors present the case report of a primigravida, aged 30 years who presented with amenorrhoea of 35 weeks. She was diagnosed as complete heart block by cardiologist. She had no history of syncopal attacks during childhood or antenatal period. She had a heart rate of 50-52 bpm. ECG showed ventricular escape rhythm with narrow QRS complexes. Echocardiography showed no structural defects. Her elective LSCS was done at 39 weeks after consultation with cardiologist. Patient underwent temporary pacemaker insertion just prior to surgery and she delivered a healthy female baby of weight 3 kg with Apgar 9/10 at 5 mins. She remained asymptomatic during the postoperative period and pacemaker was removed after 30 hours. She was discharged under satisfactory condition on day 6. Patient was counselled for follow up with cardiologist. This case lays emphasis on proper history taking, vitals monitoring, early recognition of undiagnosed cardiac disorders and team work for good maternal and fetal outcome.

scholarly journals A Rare Case of Congenitally Corrected Transposition of Great Arteries with Complete Heart Block

2012 ◽  
Vol 7 (1) ◽  
pp. 53-55
Author(s):  
Muhammed Mustafizur Rahman ◽  
Md Arifur Rahman ◽  
Md Abu Siddique ◽  
Md Khurshed Ahmed ◽  
Md Ashraf Uddin Sultan
Keyword(s):  
Heart Block ◽  
Rare Case ◽  
Complete Heart Block ◽  
Transposition Of Great Arteries ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

DOI: http://dx.doi.org/10.3329/uhj.v7i1.10212 UHJ 2011; 7(1): 53-55

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