Cardiomiopatia restrittiva

Question 1

Eziologia

NB Nella cardiomiopatia restrittiva è facilmante palpabile il polso apicale, cosa che solitamente non accade nella pericardite costrittiva.

Answer 1

• Idiopathic
• Systemic diseases (Infiltrative cardiomyopathy)
  
  1. Amyloidosis (most common cause)
  2. Sarcoidosis
  3. Hemochromatosis
• Systemic sclerosis

Other causes

• Endomyocardial fibrosis: a disorder with unknown etiology characterized by focal or diffuse endomyocardial thickening
• 👓Löffler endocarditis: a condition characterized by eosinophilic infiltration of endocardium and myocardium occurring in diseases accompanied by eosinophilia (Eosinophilia can be caused by different predisposing conditions e.g., hypereosinophilic syndrome, parasitic infection, eosinophilic leukemia). Tratta con glucocorticoidi e idrossiurea o interferone.

-Endocardial fibroelastosis: a condition characterized by diffuse thickening of the left ventricular endocardium due to proliferation of fibrous and elastic tissue
Can be primary (with unknown etiology) or secondary (associated with various congenital heart conditions such as aortic stenosis, aortic atresia, coarctation of the aorta, patent ductus arteriosus, etc.)
Most commonly occurs in the first 2 years of life

-Iatrogenic causes of myocardial fibrosis
Chemotherapy: anthracyclines (e.g. doxorubicin), alkylating agents (e.g. carboplatin, cisplatin), tyrosine kinase inhibitors (e.g. imatinib, sorafenib), monoclonal antibodies (trastuzumab)

• Radiation of the chest
• After open heart surgery

Question 2

Pathophysiology

Answer 2

Pathophysiology
Proliferation of connective tissue → ↓ elasticity of myocardium → ↓ ventricular compliance → ↓ diastolic filling → atrial congestion → atrial enlargement and severe diastolic dysfunction → systemic venous congestion

Question 3

Clinica

Answer 3

• Most common: dyspnea
• Symptoms of right heart failure
• Jugular venous distention
• Peripheral edema, ascites
• Hepatomegaly
• See “Clinical features” in CHF.
• Kussmaul sign👓
• Possibly S4 heart sound

Question 4

Echocardiography

NB atri dilatati e pareti ventricolari ispessite!👓

Answer 4

• Signs of diastolic dysfunction
• Normal or decreased ventricular volume
• Rapid early but reduced diastolic filling (questo spiega onde x e y molto negative)
• Near normal or elevated EF
• Atrial enlargement and dilation
• Wall thickening is possible (usually symmetric ).

Question 5

Endo/myocardial biopsy (gold standard)

Answer 5

• Histology classically shows fibrosis.
• Diagnosis of underlying cause if other tests are inconclusive (e.g., amyloid or iron depositions, eosinophilic infiltrates in Löffler endocarditis)

Question 6

Treatment

Answer 6

Treatment is generally limited and often only palliative.

-Treatment of underlying condition (e.g., phlebotomy for hemochromatosis)

Symptomatic treatment

-Maintenance of sinus rhythm: beta blockers
↑ Ventricular filling time, ↓ sympathetic activity: cardioselective calcium channel blockers

-↓ Preload: ACE inhibitors
Exception: ACEIs are poorly tolerated in amyloidosis.

• For fluid overload: diuretics
• Anticoagulation (e.g., warfarin) to prevent embolism in patients with a history of atrial fibrillation
• Heart transplant (in patients with refractory symptoms)

Question 7

Cateterismo cardiaco

Answer 7

Curva ventricolare diastolica con morfologia dip-plateau (non confondere con il polso venoso giugulare) o a radice quadrata. La pressione di riempimento sinistro supera di 5 mmhg quella destra accentuandosi con Valsalva, esercizio o sovraccarico di volume.