HYPERTROPHIC CARDIOMYOPATHY: Severe Heart Failure. Paolo Spirito, Genoa, Italy

Transcription

1 HYPERTROPHIC CARDIOMYOPATHY: Severe Heart Failure Paolo Spirito, Genoa, Italy

2 Clinical Substrates for Heart Failure Symptoms in HCM Diastolic dysfunction Atrial fibrillation LV outflow obstruction Evolution to end stage (EF < 50%) Combinations of the above

3 Clinical Substrates for Heart Failure Symptoms in HCM Diastolic dysfunction Atrial fibrillation LV outflow obstruction Evolution to end stage (EF < 50%) Combination of some of the above

4 Impact of LVOT Obstruction on Clinical Course in HCM P=0.002 P = P=0.001 Autore et al. JACC 2005

5 Impact of LVOT Obstruction on Survival in HCM P=0.02 Maron MS et al. NEJM 2003 Autore et al. JACC 2005

6 There is no definitive evidence that invasive treatment of LVOT obstruction increases survival in HCM.

7 Therefore, purpose of invasive treatment remains to reduce severe HF symptoms and improve quality of life.

8 Invasive Therapy for Relief of LVOT obstruction in HCM To be performed only in centers with large experience with invasive management of LVOT obstruction in HCM.

9 Invasive Therapy for Relief of LVOT obstruction in HCM To be performed only in centers with large experience with invasive management of LVOT obstruction in HCM. In Patients with: 1) Dynamic LVOT gradient > 50 mm Hg at rest or with physiologic provocation; 2) Severe and drugrefractory symptoms of heart failure (FC III or IV).

10 Invasive Treatment in Obstructive HCM Surgical Myectomy Alcohol Septal Ablation

11 Surgical Myectomy

12 4.2 cm Myectomy 5.2 gr

13 Myectomy Dearani JA et al. Nat Clin Pract Cardiovasc Med: 2007

14 Anomalies of Insertion of MV Apparatus on Ventricular Septum in HCM Dearani JA et al. Nat Clin Pract Cardiovasc Med: 2007

15 Survival free from HCMrelated death (%) SURGICAL SEPTAL MYECTOMY Myectomy Nonobstructive P=N.S Years Ommen et al. JACC 2005

16 Survival free from HCMrelated death (%) Septal Myectomy in 124 Consecutive Patients with Obstructive HCM ( ) 100 Survival free from HOCM related death Mean followup 31 months (1168) HCMrelated Mean followup mortality 31 mo. 0.9%/yr (1168) HCM related mortality 0.9%/yr 500 I I I I Years Months Cardiac Surgery, Bergamo, Italy (unpublished results)

17 Advantages and Limitations of LV Myectomy Advantages > 40 years of experience with septal myectomy Favorable longterm results reported by multiple centers Does not cause a myocardial scar Can repair abnormalities of the MV apparatus Limitations Requires cardiac surgery Requires a center with large experience with myectomy

18 Alcohol Septal Ablation

19 ALCOHOL SEPTAL ABLATION Basal 2.5 mm Balloon After 2 ml alcohol

20 ALCOHOL SEPTAL ABLATION Before After

21 Septal Alcohol Ablation in HCM: Levovist Distribution LV RV RA LA LA RA LA RA LA LV LA LA RA LA LV Papillary Muscle LV Posterior Wall RV Papillary Muscle Courtesy of Dr. Hubert Segewiss

22 SEPTAL SCARRING MRI postalcohol ablation MRI postmyectomy Septal Scar No Scar

23 56 y.o. HCM Patient, 16 Days after Alcohol Septal Ablation Ross et al. NEJM 2004

24 Appropriate ICD Interventions after Alcohol Ablation vs Myectomy (subanalysis in the Multicenter ICDHCM Study, JAMA 2007) Septal Ablation: 4/17 patients (10.3%/year) Myectomy: 6/50 patients (2.6%/year) P = 0.04

25 LongTerm Outcome of Alcohol Ablation vs Myectomy in HCM ten Cate FJ et al. Circulation Heart Failure, 2010

26 Advantages and Limitations of Alcohol Septal Ablation Advantages Performed percutaneously Limitations Causes a myocardial infarction and a scar in patients with an arrhythmogenic LV and at risk of sudden death Cannot repair abnormalities of the MV apparatus The available longterm results are scarce and not encouraging

27 CASE PRESENTATION Patient: 32 y.o. male with Obstructive HCM Clinical presentation: LVOT gradient 70 mm Hg under basal conditions; Thickness of LV anterior septum: 24 mm; LA dimension: diameter 52 mm, area 30 cm 2 ; Moderate MV regurgitation;

28 CASE PRESENTATION Patient: 32 y.o. male with Obstructive HCM Clinical presentation: LVOT gradient 70 mm Hg under basal conditions; Thickness of LV anterior septum: 24 mm; LA dimension: diameter 52 mm, area 30 cm 2 ; Moderate MV regurgitation; Mild symptoms of heart failure (FC II) on medical treatment.

29 Question: In this young patient, should we wait for the development of severe HF symptoms and AF, or should we accept the risk of surgical myectomy in an attempt to delay progression of the disease and possibly prolong survival?