A pilot-study to assess Coronary Flow reserve and post-vasodilation Myocardial Blood Flow , measured by PET with 13N-Ammonia, before and after a six-month long treatment with the ACE-Inhibitor Perindopril, in patients with moderate and high-risk Hypertrophic Cardiomyopathy

Phase 1

• Conditions: Hypertrophic Cardiomyopathy (primitive); MedDRA version: 20.0Level: PTClassification code 10020871Term: Hypertrophic cardiomyopathySystem Organ Class: 10010331 - Congenital, familial and genetic disorders; Therapeutic area: Diseases [C] - Cardiovascular Diseases [C14]

• Registration Number: EUCTR2015-004317-24-IT
• Lead Sponsor: AZIENDA OSPEDALIERO-UNIVERSITARIA CAREGGI

Brief Summary

Not available

Detailed Description

Not available

Study Timeline

• Results First Posted: 1900-01-01
• Study Start: 2021-09-09
• Last Update Posted: 8 October 2021

Recruitment & Eligibility

• Status: Authorised-recruitment may be ongoing or finished
• Sex: All
• Target Recruitment: 21

Inclusion Criteria

1. Age in the range 18-60
2. Availability of a 12-lead rest-ECG, performed not
earlier than 60 days prior to enrollment visit (EV).
This is called B-ECG (Baseline-ECG), in the following
text.
3. Availability of a 2-D Doppler echocardiogram,
performed at rest, not earlier than 60 days before of
EV. This is called B-ECO (Baseline - Ecocardiography in the following text.
4. Availability of a 13N-NH3 PET/CT myocardial
perfusion scan for measurement of Myocardial Blood
Flow (MBF), global and regional, performed not
earlier than 60 days before of enrollment visit, at
one or the other of involved clinical trial center. This
is called B-PET/CT (Baseline-Positron Emission
Tomography/Computed Tomography), in the
following text
5. Availability of lab tests (including at least:
a. Hematocrit and complete blood count (CBC)
b. blood urea nitrogen (BUN)
c. serum creatinine,
d. blood glucose,
e. serum potassium ion
performed not earlier than 60 days before of
enrollment visit. This is called Baseline-Blood Tests”
(B-BT), in the following text.
17
6. Fulfillment of 2D-echocardiographyc (as assessed by
B-ECO), and clinical criteria for diagnosis of HCM
according to the ACCF/AHA Guideline (2011) for
diagnosis of HCM:
a. maximum LV wall thickness >1.5 cm
b. not-dilated LV cavity
in the absence of clinical evidence for any other
cardiovascular or systemic etiology, justifying the
degree of myocardial hypertrophy actually seen
(e.g., arterial hypertension, aortic valve stenosis,
cardiac amyloidosis, metabolic storage diseases, as
detailed in the exclusion criteria).
7. Absence of severe, resting LV outflow tract
obstruction (peak gradient, measured at rest, >50
mmHg), as assessed by B-ECO.
8. Global Post-Dipi gMBF < 2.1 mL/min*gr, as assessed
by 13N-NH3 PET/CT myocardial perfusion scan at
baseline (B-PET/CT).
9. Stable sinus rhythm, as assessed by B-ECG.
10. Clear ability to understand the significance and the procedures of the Protocol and to sign a legally valid
informed consent.

Are the trial subjects under 18? no
Number of subjects for this age range:
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range 21
F.1.3 Elderly (>=65 years) no
F.1.3.1 Number of subjects for this age range

Exclusion Criteria

1. Women, who are pregnant or lactating
2. Women, i in childbearing age, not using an adequatecontraception (i.e., techniques having Pearl index<2)
3. Clinical evidence or previous clinical story of diseasescausing myocardial hypertrophy or similar
phenotypes:
a. Aortic stenosis
b. Cardiac amyloidosis
c. hemochromatosis
d. storage diseases
4. Coronary Artery Disease :
a. previous coronarography, assessing 1 or
more stenosis >70% of major epicardial
arteries
b. Clinical story for previous PTCA
c. Clinical story for previous CABG.
5. Evidence for fixed or inducible regional defect of
perfusion of LV, as assessed by B-PET/CT,
suggesting CAD, unless recent (<30 days old)
coronarography, negative for stenosis >70% in any
of the major epicardial arteries, is available.
6. Evidence for valvular heart disease (particularly for
aortic stenosis), as assessed by B-ECO.
7. Previous clinical story for arterial hypertension,
requiring antihypertensive medication
8. Arterial pressure >145 mmHg systolic and/or >90
mmHg diastolic measured at EV
9. Arterial pressure <110 mmHg systolic measured at EV (Enrollment Visit)
10. LV outflow tract obstruction, with peak of ejection
gradient >50 mmHg measured at rest, as assed by
B-ECO
11. Prior surgical septal myectomy or alcoholic ablation;
12. LV systolic dysfunction with EF <50% as assessed
by B-ECO.
13. Evidence for electric ventricular conduction
abnormalities such as LBBB, RBBB, WPW, AV blocks,Long QT, as assessed by B-ECG.
14. Body mass index >32 Kg/m2
15. Clinical evidence or clinical story for Diabetes
Mellitus
16. Clinical story for vasculitis or connective tissue
disease (AR, LES, SS, MCTD, Churg Strauss
vasculitis, ANCA-linked vasculitis, polyarterits
nodosa, temporal arteritis, Takayasu disease, etc.)
17. Clinical story for cancer of any kind unless nonmelanoma skin cancers
18. GFR< 60 mL/min estimated by MDRD equation and by B-BT data.
19. Clinical story for chronic liver disease with
insufficiency > class A Child-Pugh
20. Present evidence or previous story of neurologic
diseases (cerebrovascular, neurodegenerative,
demielinating)
21. Clinical story for psychiatric disorders or
administration (previous or present) of psychotropic
drugs (unless minor tranquillizers or hypnotics)
22. Clinical story for psychotropic drugs abuse or
addiction, or for alcohol abuse/addiction
23. Any contraindication to the administration of
Perindopril,such as:
a. Hypersensitivity to Perindopril or other
ACE-inhibitors
b. Clinical story for previous angioedema due
to administration of ACE-inhibitors
c. Clinical story for idiopathic or hereditary
angioedema
d. Aortic valve stenosis
e. Mitral valve stenosis
f. Liver failure > class A Child Pugh.
g. GFR<60 ml/min, as estimated by MDRD
equation and B-BT data
h. LV outflow tract obstruction in HCM with
peak of ejection gradient >50 mmHg
measured at rest (assessed by B-ECO)
i. Concurrent therapy with diuretics
j. Concurrent therapy with potassium
supplements or potassium sparing diuretics
k. Concurrent therapy with lithium salts
24. Any contraindication to the administration of
Dipyridamole (Persantyn®), particularly:
a. Individual hypersensitivity to the active
substance or excipients of medicinal product
b. Severe asthma
25. Any contraindication to the administration of
Aminophilline (Aminomal®), particularly:
a. Individual hypersensitivity to the active
substance or excipients of the medicinal
product (Aminomal®)
b. Individual hypersensitivity to other
methylxanthines, and

Study & Design

• Study Type: Interventional clinical trial of medicinal product
• Study Design: Not specified