Congenital complete atrio-ventricular bock and dilated cardiomyopathy - new light for an old disease

• Type: Summary of Presentation in an International Conference
• Date: 2012

Authors

Paiva M (Author) Other; Ribeiro V (Author) Other; Sousa A (Author) Other; Garcia R (Author) Other; Vieira A (Author) Other; Amorim S (Author) Other; Martins E (Author) Other; Campelo M (Author) Other; Silva Cardoso J (Author) Other; Maciel MJ (Author) FMUP

Conference proceedings International

Title: European Journal of Heart Failure Supplements

Initial page: P447

Heart Failure Congress 2012

Belgrade, 2012

Scientific classification

FOS: Medical and Health sciences > Clinical medicine

Other information

Abstract (EN): A 7 years-old Caucasian boy was sent, in 1997, to a pediatric cardiology outpatient clinic, due to a slow heart rate and exercise intolerance. His EKG showed sinus rhythm, 44 bpm, complete atrioventricular block (CAVB) and left bundle branch block. His TTE showed no structural abnormalities. Exercise treadmill test revealed an inappropriate chronotropic response to exercise. The 24 hour Holter test confirmed permanent CAVB and the absence of other abnormalities. At age 11, for worsening of exercise tolerance, a VVI-R pacemaker was implanted. He was an active child and, after VVI-R implantation, he had no exercise limitations. Again, TTE showed no relevant abnormalities. At age 15, while completely asymptomatic, TTE showed a new mild left ventricular systolic dysfunction (LVSD), EF=46%. At age 18, due to battery depletion, the pacemaker generator was replaced. By 19 years-old, an engineering student, he was in NYHA class I, TTE showed a severe LVSD, with slight increase of LV diameter and mild right ventricular systolic dysfunction; he started lisinopril and bisoprolol. By age 21, he became symptomatic with intense dyspnea, irritative cough and orthopnea. In the emergency room he was in NYHA class IV and hypotensive. BNP and D-dimers levels were high. The EKG was similar to previous ones and the TTE showed severe right chamber dilatation, severe bi-ventricular dysfunction, severe tricuspid regurgitation and estimated PASP of 62 mmHg. A chest CT-scan showed signs of chronic pulmonary embolism. Full dose enoxaparin was started and he was hospitalized. He improved clinically, although maintaining mild to moderate exertion dyspnea. TEE showed no left atrial appendage thrombus and the genetic pro-thrombotic analyses revealed a deficiency of C-protein. As he was life-long pacemaker dependent, with severe bi-ventricular dysfunction and symptomatic despite medical therapy, VVI-R was upgrade to CRT-D. QRS complex became narrower (from 174 to 126 mseg). He was discharged on candesartan, bisoprolol, spironolactone, warfarin and furosemide. One month after, in NYHA class II, TTE showed a slight improvement in left ventricular function, while maintaining right chamber dilation and pulmonary hypertension (PASP 67 mmHg). Congenital CAVB may develop DCM. The recognition of right ventricular pacing deleterious effects may suggest the implantation of bi-ventricular pacing in some selected cases. In this case, the decision to upgrade to a CRT-D was controversial and discussion about the best pacemaker system will continue.

Language: English

Type (Professor's evaluation): Scientific

Notes: Paiva M, Ribeiro V, Sousa A, Garcia R, Vieira A, Amorim S, Martins E, Campelo M, Silva Cardoso J, Maciel MJ. Congenital complete atrio-ventricular bock and dilated cardiomyopathy - new light for an old disease. Eur J Heart Fail Suppl 2012; 11(Suppl 1): 447. ISSN 1567-4215. Heart Failure Congress 2012, Belgrade, 2012