BRIEF COMMENTS
EXPERT: Prof. Janusz Skalski MD, PhD
Affiliation: Department of Pediatric Cardiac Surgery, Jagiellonian University Medical College, Krakow
COMMENT
Diagnosis of cardiomyopathy needs closer evaluation. Ventricular hypertrophy and pulmonary arterial hypertension might be consequences of congenital heart defect, not necessarily cardiomyopathy. It is not clear why the patient wasn’t operated in the past?
EXPERT: Prof. Katarzyna Mizia-Stec MD, PhD
Affiliation: 1st Department of Cardiology, Medical University of Silesia, Katowice,Poland
COMMENT
Ventricle hypertrophy was first noticed in 2005. Question is if the pulmonary hypertension was present at the beginning? This might have driven the decision against the surgical correction of the defect. Could such a significant hypertrophy be caused by the congenital heart disease? The patient was put on bosentan and his clinical symptoms and laboratory results has been gradually improving since then.
EXPERT: Jacek Pająk MD, PhD;
Affiliation: Silesian Center for Heart Diseases, Zabrze, Poland
COMMENT
In case of wide VSD, pressure in both ventricles might be equal so the thickness of the walls of both ventricles will be similarly enlarged. Diagnosis of the cardiomyopathy should not be neglected, but in my opinion this hypertrophy is the consequence of congenital heart defect.
EXPERT: Grzegorz Kopeć MD, PhD
Affiliation: Department of Cardiac and Vascular Diseases, John Paul II Hospital, Krakow, Poland
COMMENT
From my observation, Down patients with VSD often have hypertrophied left and right ventricle walls LV diastolic dysfunction is also often seen.
EXPERT: Lidia Tomkiewicz-Pająk MD, PhD
Affiliation: Department of Cardiac and Vascular Diseases, John Paul II Hospital, Krakow, Poland
COMMENT
We had similar patient in our observation –LVOT obstruction and bradycardia were present, pacemaker was implanted and beta blocker was administered – we observe gradient reduction and clinical improvement.
EXPERT: Prof. Lesław Szydłowski MD, PhD
Affiliation: 1st Department of Paediatric Cardiology, Medical University of Silesia, Katowice-Ligota, Poland
COMMENT
There may be coincidence of congenital heart defect and cardiomyopathy – I never observed such hypertrophy in VSD patients.
CONCLUSIONS
1) Both ventricular hypertrophy probably caused by congenital heart disease
2) Diagnosis of the hypertrophic cardiomyopathy requires reevaluation
3) Bosentan therapy continuation is recommended
“Development of the European Network in Orphan Cardiovascular Diseases”
„Rozszerzenie Europejskiej Sieci Współpracy ds. Sierocych Chorób Kardiologicznych”
