Dilated Cardiomyopathy: Everything that you should know
DCMP is a chronic heart muscle disease where there is dilatation and systolic impairment of the Left or both ventricles (pumping chambers of the heart ) unexplained by abnormal loading conditions or coronary artery disease sufficient to cause the observed myocardial dysfunction. Basically, there is systolic dysfunction (low ejection fraction with normal LV wall thickness and LV dilatation.
Dr Hemant Pathare, Consultant Cardiovascular Surgery, Jaslok Hospital and Research Centre shares that some common symptoms are fatigue, breathlessness, palpitations or swelling of feet which are non-specific initially.
A. Genetic DCMP (More than 60 per cent are inherited. prevalence is 1:2500)
B. Non-genetic DCMP
1. Auto immune DCMP seen in Rheumatoid Arthritis. Systemic Lupus
2. Penpertum DOMP seen in last month of pregnancy or 5 months postpartum: Essentially a diagnosis of exclusion. (0.1% of all pregnancies). 75% of cases diagnosed in 1st month after delivery. risk factors age > 30 yrs, chronic
hypertension, preeclampsia, multiparity essentially
3. Alcoholic CMP is seen in habitual heavy alcohol consumption (>3 pegs daily)
4. Cirrhotic CMP due to liver failure and portal hypertension
5. Toxic CMP d/t chemotherapy and radiotherapy for cancers Doxorubicin/
Cyclophosphamide/ Bevacizumab
Specialised Investigations include a 2D echocardiogram, a Coronary angiography r/ o coronary atherosclerosis and cardiac MRI which will reveal hyper trabeculation or non-compaction. A NT-Pro-BNP test and a VO2 max quantify the degree to heart failure.
Medical management is the initial treatment.D different classes of drugs are available which are used in judicious combinations including Diuretics ~ Frusemide, Angjotensin Converting Enzyme inhibitors like Ramipril, Angjotensin Receptor Blockers viz Telmasartan ARNIs eg, Saccubitril valsartan, Beta blockers eg. Carvidilol and anti aldosterone therapy eg. Eplerenone . The next step involves CRT and ICDs.
Cardiac Resynchronisation (CRT) therapy allows a specialised pacemaker to make both the ventricles contract in unison to augment cardiac output. An Implantable Cardioverter Defibrillator (ICD) terminates fast Ventricular rates and abnormal rhythms.
Surgical management includes LVADs and Heart transplantation
End-stage heart failure affects >2+ million people worldwide and because of an acute shortage of donor's hearts, many older patients will die whilst being waitlisted on the heart transplant list.
Left Ventricular Assist devices
AWAD is a mechanical pump that takes over some or all of the pumping function of the heart. Usually, all patients listed for a heart transplant are fit for a LVAD/bi VAD. Additionally, certain patients in whom a heart transplant is contradicted may be acceptable candidates for destination therapy with LVAD’s . Essentially a LVAD pumps blood from the left side of the heart to the aorta in a continuous manner whilst an RVAD pumps blood from the right side of the heart to the pulmonary artery for treating right ventricular failure. a BiVAD is used to treat bi-ventricular failure. The VAD pump is run by a small external controller which is connected to it by a driveline which passes through the skin of the upper abdomen or behind the ear & is powered by Zz rechargeable batteries. The complete system weighs approx Lg kes. VADS can be either bridge to transplant or destination therapy or as a bridge to decision. VADs are silent during operation but can pump unto 10 litres/minute. postoperatively the patient usually stays in NCU for 5 days & 2 weeks in the hospital ward. Thereafter the patient remains on blood thinner viz anticoagulants (Warfarin) and antiplatelet agents (Aspirin) lifelong. Once the patient is confident of managing the controller, he/ she is discharged for a regular follow up in the heart failure clinic. Most of the heart failure in the western world are bridged to a heart transplant with a VAD, commonly used VADS and HVAD, Jarvik and Heartmate III.
A heart transplant involves replacing the diseased heart with a compatible heart. This prolongs the life of patients and gives them excellent quality of life. Currently, survival rates are 70% at 5 years with a median survival of 14 years.
Total artificial heart: Syncardia TAh is usually utilized as a bailout procedure for hyperacute heart rejection after a heart transplant.