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Tesi etd-11222019-112740


Thesis type
Tesi di specializzazione (5 anni)
Author
SAPONARA, GIANLUIGI
URN
etd-11222019-112740
Title
PROGNOSTIC ROLE OF PULMONARY HYPERTENSION IN NON OBSTRUCTIVE HYPERTROPHIC CARDIOMYOPATHY
Struttura
PATOLOGIA CHIRURGICA, MEDICA, MOLECOLARE E DELL'AREA CRITICA
Corso di studi
MALATTIE DELL'APPARATO CARDIOVASCOLARE
Supervisors
relatore Prof. Pedrinelli, Roberto
relatore Dott.ssa Frigerio, Maria
Parole chiave
  • heart transplant
  • heart failure
  • hypertrophic cardiomyopathy
Data inizio appello
18/12/2019;
Consultabilità
Secretata d'ufficio
Data di rilascio
18/12/2089
Riassunto analitico
Pulmonary hypertension is associated with increased mortality in all forms of left-sided hearth disease.The aims of this study was investigate the prevalence and clinical implications of pulmonary hypertension in patients with non-obstructive HCM and heart failure. HCM is the most common inherited/genetic hearth disease and occur in 1 of every 200/500 persons. The long term prognosis of HCM, especially thanks to the primary prevention of sudden death with ICD, could be comparable to that of general population. In about 35-40% of HCM cases left ventricle outflow tract obstruction (LVOTO) is not found at rest or provoked, in these patients the risk of developing HCM-related complications is lower. About 10% of patients with non-obstructive HCM develop severe functional limitation and becomes refractory to medical management due to the onset of systolic dysfunction, severe diastolic dysfunction or due to the combination of the two phenomena. In this cases heart transplantation is the only effective therapy. Methods and Results:The study population consisted of 96 patients with non obstructive HCM consecutively referred between 1991 and 2019 to 3 italian centers and retropsectively assessed. Clinical, echocardiographic and right heart catheterization data were collected at the time of index visit and we divided patients in two groups: group NOPH (A) PAmP at RHC <25 mmHg (n=38) and group PH (B) PAmP at RHC >=25 mmHg (n=58). Discussion: This study explores the prevalence and clinical impact of pulmonary hypertension (detected with RHC) in patients with non-obstructive HCM and heart failure symptoms mainly referred to our centers to perform cardiac transplant assessment. The rate of events is high and the median time between the index evaluation and the composite endopoint is 1.6 years. The presence of pulmonary hypertension at the index evaluation is associated with a higher risk of events, intended as a composite end-point of LVAD cardiac transplant and death, while no post-transplant prognosis differences between the two groups of patients have emerged, although in patients with pulmonary hypertension there was a higher rate of death due to post Tx / LVAD complications. PAPs echocardiographically calculated results in a good percentage of cases not executable or poorly reliable if compared with the catheterization data.
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