​Kembara Xtra - Medicine- Cardiomyopathy

​Kembara Xtra - Medicine- Cardiomyopathy  
Introduction 
In the absence of coronary artery disease, congenital heart disease, valve disease, or hypertension that could adequately explain the clinical myocardial dysfunction, cardiomyopathies are myocardial disorders that cause structural and functional heart abnormalities.

With the current classification system, it is possible to distinguish between cardiac diseases that are limited to the myocardium (primary) and those that are secondary to systemic problems. A third, distinct category is given to specific causes of myocardial dysfunction brought on by other cardiovascular diseases.
Primary cardiomyopathies are those that primarily affect the myocardium.
The genetic
Cardiomyopathy with hypertrophy (HCM)
Left ventricular (LV) noncompaction (LVNC), arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), and more
Storage of glycogen (Danon type, PRKAG2)
Conductivity issues
Ion channel diseases, including long QT syndrome (LQTS), Brugada syndrome, short QT syndrome, and catecholaminergic polymorphic ventricular tachycardia (CPVT), include mitochondrial myopathies. Mixed (non-genetic and genetic)
Restrictive (nonhypertrophied and nondilated) dilated cardiomyopathy
Myocarditis, stress cardiomyopathy, peripartum, tachycardia-induced birth defects, and children of type 1 diabetes moms are acquired conditions that are secondary (multiorgan involvement; see list below).
 Particular: congenital heart disease, hypertension, ischemia, and valvular heart disease 

End-stage cardiomyopathy patients have stage D heart failure or significant symptoms at rest that are resistant to conventional medical treatment.
cardiovascular, renal, hepatic, and pulmonary systems are impacted Child Safety Considerations
Early diagnosis and a progressive course may change the course of the disease. causes include endocrine, uremic, nutritional, childhood LVNC, DCM, HCM, and RCM. pregnant women's issues
Peripartum cardiomyopathy (PPCM) can happen months before and after delivery in peripartum women.


Prevalence and incidence of disease 

The most common cause of ischemic cardiomyopathy, which affects people mostly over 50 years old, is aging. Think about unusual reasons in children. HCM is a significant underlying cause of heart failure impairment and the most frequent cause of sudden cardiac death in young people.
Incidence
 DCM: 5–8 new cases per 100,000 people each year.

Prevalence
At least 1 in 500 adults have DCM, which is the third most common cause of heart failure and the most common reason for heart transplantation. Tropical regions are more likely to experience RCM.

Pathophysiology 
LVNC: congenital cardiomyopathies with "spongy" appearance of the LV myocardium; HCM: hypertrophied, nondilated left ventricle; ARVC/D: involves the right ventricle with progressive loss of myocytes and fatty/fibrofatty tissue replacement; can be associated with myocarditis (adenovirus or enterovirus); LQTS: most common ion channelopathy with prolonged ventricular repolarization and QTc RCM is defined as normal/decreased ventricular volume with restrictive physiology, enlarged biatrial chambers, and impaired ventricular filling. Myocarditis is defined as an acute or chronic inflammation of the myocardium caused by toxins, drugs, or infectious causes. PPCM is defined as a form of DCM with LV systolic dysfunction and heart failure of unknown etiology. Stress cardiomyopathies are defined as acute but quickly reversible  Viral infections such as HIV, coxsackievirus, and adenovirus are infectious causes.
- Mycobacterial and bacterial diseases (such as diphtheria and rheumatic fever). - Parasitic (such as trypanosoma cruzi and toxoplasmosis) 
Neuromuscular/neurologic: Duchenne and Emery- Dreifuss muscular dystrophies, Friedreich ataxia, myotonic dystrophy, neurofibromatosis, tuberous sclerosis; Infiltrative: amyloidosis, Gaucher disease, Hurler disease, Hunter disease, Fabry disease; Storage: hemochromatosis; Glycogen storage disease (type II, Pompe); Heavy Metal; Chemic; Neuromuscular/neurologic: Sarcoidosis is an inflammatory condition with granulomatous symptoms. Idiopathic conditions include endomyocardial fibrosis and hypereosinophilic syndrome (Loeffler endocarditis).
Genetics
The most prevalent kind of primary hereditary cardiomyopathy, autosomal dominant HCM, is brought on by many mutations in the genes encoding the contractile proteins of the heart sarcomere. Only one-third of DCM instances have genetic origins; these cases are almost exclusively inherited autosomally dominantly. Along with LQTS and other ion-channel abnormalities, LVNC and ARVC are inherited in an autosomal dominant manner as well.

Risk factors include smoking, physical inactivity, excessive alcohol use, dietary salt, obstructive sleep apnea, diabetes mellitus, hypertension, hyperlipidemia, obesity, coronary artery disease, and chemotherapy.

Prevention 
Limit your intake of salt and water, and measure your daily weight and BP at home.


Presenting History: Right upper quadrant pain or bloating; orthopnea; postprandial dyspnea; dyspnea with activity; paroxysmal nocturnal dyspnea; fatigue; syncope; edema


Tachypnea, Cheyne-Stokes breathing, low pulse pressure, cool extremities, jugular venous distention, bibasilar rales, tachycardia, displaced point of maximum impulse (PMI), S3 gallop, blowing systolic murmur, hepatosplenomegaly, ascites, and edema are all signs of a clinical condition.


Differential diagnoses include anemia, recurrent pulmonary embolism, constrictive pericarditis, severe pulmonary illness, primary pulmonary hypertension, and several advanced types of cancer.


Diagnostic tests and laboratory results 
ECG: indications of a previous Q-wave infarction, atrial fibrillation, interventricular conduction delay, and LV hypertrophy 
Prerenal azotemia Hyponatremia
Blood loss

Mild hyperbilirubinemia; mild elevation of troponin; elevated B-type natriuretic peptide (BNP) or pro-BNP; elevated liver function tests; and elevated uric acid
 
Chest radiograph - Cardiomegaly - Increased vascular markings to the upper lobes - Initial tests (lab, imaging) - ECG - There may or may not be pleural effusions.
Global hypokinesis and four-chamber enlargement are signs of DCM, according to echocardiography.
- Severe LV hypertrophy is seen in HCM.
– A past localized myocardial infarction is indicated by anomalies in the LV's segmental contraction.
Cardiovascular magnetic resonance imaging (cardiac MRI) may be helpful in identifying specific nonischemic cardiomyopathies. Stress myocardial perfusion imaging (MPI) is advised for people who have recently developed LV dysfunction or when ischemia is suspected.

Pulmonary artery catheters may be reasonable in patients with refractory heart failure to help direct care. Diagnostic Procedures/Other Cardiac Catheterization - Helpful to rule out ischemic heart disease - Characterize hemodynamic severity.

treatment management procedures.
Treating electrolyte abnormalities Lowering filling pressures

The First Line of Medicine
Systolic failure syndromes - Initiate at low dosages and titrate as tolerated to target doses; either an ACE inhibitor or an ARB is equally efficacious and should be considered in all patients.
- Sacubitril/valsartan (Entresto), a combination medication including a neprilysin inhibitor and valsartan (ARNI), was approved in 2015 as an alternative to an ACE/ARB for the treatment of systolic heart failure (ejection fraction [EF] 40%).
– Morbidity and mortality in patients who have been stable on an ACE or ARB may be further decreased by switching to an ARNI, with careful monitoring required for hypotension and angioedema (all cause mortality reduction around NNT 33 over 2 years of treatment).
Diuretics with loops
May need to be administered initially by IV and later orally if the patient is stabilized.
- 40 to 120 mg/day or TID of furosemide 
A blockers
In cases of abrupt decompensation or poor cardiac output, use with caution.
Start with small doses and increase as tolerated.
- Bisoprolol, 1.25 to 10 mg/day; metoprolol succinate, 12.5 to 200 mg/day; or carvedilol, 3.125 to 25 mg BID (3)
[A] - Aldosterone antagonists, such as spironolactone or eplerenone - Digoxin, 0.125 to 0.250 mg/day for symptomatic patients on conventional therapy for New York Heart Association (NYHA) II to IV heart failure, EF 35% (3)[B]
- For all patients with reduced EF and symptoms that are only partially responsive to ACE inhibitors and beta-blockers, combination hydralazine/isosorbide dinitrate is the first-line treatment for African American patients with classes III and IV symptoms who are already receiving conventional medication.
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Contraindications
Blockers: second- or third-degree heart blocks, reduced cardiac output 
Verapamil and diltiazem should not be used in patients with systolic dysfunction.
Aldosterone antagonists: renal dysfunction, oliguria, and anuria 
Hypokalemia and hypomagnesemia caused by loop diuretics
ACE inhibitors: angioedema and pregnancy
Patients using ACE inhibitors now or within the previous 36 hours, as well as those with a history of angioedema, should take ARNIs. 
Safety measures
Digoxin dose in individuals with chronic kidney disease should be kept to less than 0.125 mg per day, and medication levels should be closely monitored to prevent toxicity.
- Keep an eye on electrolytes.
- ARNIs and ACE inhibitors: Start with caution if blood pressure is low.
Blockers should be avoided in patients who have signs of poor tissue perfusion since they could further impair systolic performance.
- Cilostazol, NSAIDs, and Glitazones are medications to avoid. Milrinone and dobutamine are also drugs to avoid.

Second Line: Continuous inotrope infusion in stage D outpatients for symptom control in those who are ineligible for transplantation or mechanical circulatory support. Inotropic therapy (e.g., dobutamine or milrinone) for cardiogenic shock and support prior to surgery or cardiac transplantation.


A heart failure team's factors for referral management improve results and enable early transplant referral.

Further Treatments
For individuals with a left ventricular ejection fraction (LVEF) 35% and mild to moderate symptoms, a prophylactic implanted cardioverter-defibrillator (ICD) should be taken into consideration.

Patients in sinus rhythm with a QRS >150 ms, LVEF 35%, in functional class (FC) I to III, and ambulatory FC IV patients should be given cardiac resynchronization therapy (CRT) consideration.
Patients with severe, refractory heart failure who have no realistic chance of recovering shouldn't be given the option of getting an ICD. In such cases, palliative care is a viable alternative.
In some stage D patients, it is reasonable to consider an LV assist device as "permanent" or destination therapy or heart transplantation.

Constant Care 
fluid restriction, low-fat, low-sodium diet

Prognosis In NYHA FC IV, 20–40% of patients pass away within a year. One-year survival after a transplant is up to 94%.

Congestive heart failure complications include syncope, renal failure, arrhythmias, or sudden death