CMP

Question 1

Correct QTc =

Answer 1

QT interval/(R-R squared)

Question 2

DcM - how common, causes, what is it, présentation, Ecg, Xray, tt, prognosis

Answer 2

60% of CMD

-idiopathic -myocarditis -genetic (DMD) -chemo

Present with poor feeding, tachypnea with Resp distress (congestion), diaphoresis during feeding, FTT. Older children exercise intolerance plus pallor, low peripheral pulses, quiet heart sounds, hepatomegaly and tender liver. Ecg: tachycardia, biventricular enlargement and ST abnormalities. Tx cardiomedgaly and venous, congestion.ECHO: normal structure but biventricular/atrial dilation, thrombi!!!

Acute TT: fluid restriction, diuretics and milrinone (vasodilator and increased ionotropy) in severe cases. Chronic Tt: ACE inh (increase CO, decrease LEDP), aspirin for thrombi. Cardiac transplantation for refractory patients, prognosis poor.

Question 3

HCM

Answer 3

30% of CMD

-Hypertension, CHD, diabetic mother, noonan (genetic disorder aut dom), beckwith-weidmann (overgrowth syndrome), familial. Familial type: aut dom, no other cause just primary cardiac disease, genes encoding contractile proteins, LVOTO (aortic stenosis).

So systolic and diastolic dysfunction. Differentiation between familial an athletes heart: family history of sudden cardiac death or HCM, regional differences in hypertrophy in HCM, stop exercised regression of hypertrophy.

Presentation: heart failure with tachypnea and poor feeding/ older children have chest pain and palpitations and syncope.

Examination: normal heart sounds. EcG: LVH +q waves, stt abnormalities, arrhythmia stress test. Echo : LVH diffusé or segmental and septal thickening, LV cavity size decrease. Diagnosis is histopathology: myocyte disarray.

Tt: drugs that decrease contractility and increase filling so beta blockers and CCB, with prophylaxis endocarditis, implantable cardioverter defibrillator for those with high grade arrhythmia to prevent SCD. Prognosis not good in those symptomatic in 1st year, better in older children without symptoms but 2-6% die annually without warning

Question 4

RCM

Answer 4

Deposits in myocardium and fibrosis in endocardium causing R and L failure. SO VENTRICLES DON’T CONTRACT, BLOOD REGURGITATION, ATRIA ENLARGE.

Presents with (R sides failure : edema, raised venous pressure, hepatomegaly and tricuspid regurgitation) (L sided failure: mitral insufficiency), cardiomegaly on Xray, RA/LA enlargement on Ecg (P waves in v1v2) and stt wave changes and AV blocks (atrial enlargement). Echo: atrial enlargement +/- ventricular reduction (ventricle doesn’t contract properly, less elastic so compliance less so atria enlarge with back flow), av valve leakage, THROMBI.

TT : ANTICOAGULANT, and SUPPORTIVE (HF)…. Cardiac transplant quick

Question 5

ARVC

Answer 5

Arrythmogenic right ventricle Cm. Fibro fatty replacement of RV myocardium. High risk for ventricular arrhythmia and sudden death. On Ecg, see blocks and epsilon waves and RV hypertrophy in right precordial leads. Tt arrythmias