Six months later was hospitalized for classical LVF -PND / orthopnoea - which rapidly progressed to class IV - with systemic venous congestion -

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2 HISTORY 48 years female, on Metformin with history of Diabetes Mellitus was diagnosed to have asymptomatic LV Dysfunction (with normal CAG) during pre-operative evaluation - for scapular injuryin RTA. A diagnosis of Non- ischemic cardiomyopathy was made and discharged on appropriate medication of beta-blockers, ACE I, Diuretics. Being female note on obstretic history and cardiovascular health during pregnancy, would have helpful. Should we stop further investigation and be satisfied with diagnosis of Non ischemic cardiomyopathy.

3 Six months later was hospitalized for classical LVF -PND / orthopnoea - which rapidly progressed to class IV - with systemic venous congestion - Elevated JVP, dependent pitting edema of both lower limbs equally which was confirmed by further Biochemical (NT ProBNP> 2000 Pg/ml) Echo Doppler study- showrd non hypertrophic ventricles and dilated LV cavity with poor LV systolic function (LVEF 33.6%) and RV dysfunction (TAPSE 1.7)

4 HOSPITAL COURSE Hospital course complicated by- Pulmonary embolism (with pulmonary infract on CT chest)- after which IV heparin started Anemia - with low serum iron and markedly increased serum folic acid(> 2000ng/ml) Pulmonary problem -Miliary mottling of both lung fields on HRCT. Inspite of all aggressive medical therapy during hospital stay, she died suddenly on 9th day of admission. In short a case of Heart Failure Progressed from stage B to D in less than six months with clinical/ laboratory evidence of multiple system involvement.

5 ETIOLOGY OF NON ISCHEMIC LV DYSFUNCTION Identifying etiology of Non ischemic LV dysfunction is important, as prognosis and therapy markedly different between various types of dilated cardiomyopthies. Does course of disease give us clue to etiology? The usual case of DCM is insidious onset with slow progression over a period of 5 10 years with survival of 50% at 5years. The patients had rather fulminant course causing death in less than 1year indicative of myocarditis.

6 The challenge of finding correct etiology in patients with CHF is illustrated by studies that compared clinical diagnosis with morphological diagnosis at cardiac transplant. In two large series, spanning two decades each. the percentage of patients with difference in diagnosis - between clinical and morphological - varied from 13-17% - particular with patients in non-ischemic cardiomyopathy % - (approximately one in four being wrongly labeled). The conditions that were missed clinically included myocarditis sarcidosis and non-compaction of LV. In one series of 1230patients, labelled as dilated cardiomyopathy only 50% had idiopathic DCM and 21% had either myocarditis (9%),infiltrative diseases (5%), peripartum cardiomyopathy (4%) and connective tissue disorder (3%), other miscellaneous conditions - HIV infection substance abuse and drug induced or aggravated by oncochemotherapy, forming rest etiology.

7 Diabetes Mellitusis present approximately 15-20% of patients with CHF. Adverse impact of DM on Heart is much more in females. The time from onset of DM to development of CHF is 8.5 years in males to 12.6 years on females.the exact mechanism of Heart Failure in DM is multifactorial.

8 HISTORY AND EXAMINATION Historical and physical exam contribute little to etiological diagnosis of Heart Failure, Acute heart failure after an antecedent flu like illness is suggestive of myocarditis just as alcoholic abuse is suggestive of alcoholic DCM. Similarly past history of prolongedoncochemotherapy - like drugs, for childhood malignancy, might manifest as CHF induced by drugs. during adulthood /Few extra cardiac findings may lead to specific etiology but these are infrequent but should looked for - like peri orbital purpura (for AL type of Amyloidosis) and rare triad of DM, Skin pigmentation and cirrhosis- Hemochromatosis. Involvement of other organs- Skin (Erythema Nodosumin Tuberculosis), Lung (Sarcoid/Pulmonary TB) and Intestine should be carefully looked for etiological clues. When multisystem involvement including Heart occur, as in this patient- infection (Viral/Protocol) infiltrative / degenerative disorder, and vasculitis should be seriously considered.

9 This patients ECG showed sinus rhythm with no evidence of hypertrophy. Low voltage ECG complex should also alert to you, possibility of hypothyroidism, with its adverse impact upon cardiovascular system. Does ECG contribute to etiological diagnosis? This patient had severe rhythm, low voltage in limb( <5mm) and <10mm in pericardial leads) with no significant ST/ T changes.integration of Physical findings with ECG could give clues to etiology of DCM, for example - evidence of LVH - by physical exam with low voltage in limb leads is typical of idiopathic DCM. Just as amyloidosis - where low voltage is seen in both limb and percardial leads. A heart block / AV conduction disturbance is a feature of Infiltrative disorder - Sarcoidosis/ Tuberculosis. Atrial fibrillation with rapid ventricular rate in absence of valvular heart disease, specially in young patients should alertone to possibility of Tachy induced cardiomyopathy.

10 INVESTIGATIONS Blood chemistry in patients with CHF more often reflect consequences of CHF on various organs rather than etiology of CHF. Some of the data may offer prognostic indicators like Hyponatremia and markedly elevated NT- ProBNP with poor response to therapy and severely deranged LFT (indicative of ischemic hepatitis rather than of passive venous congestion). This patient had persistent hyponatremia and progressive worsening of renal function(marker of progressive worsening of cardiac output), indications poor prognosis.

11 Occurrence of anaemia in CHF like in this is patient is also marker of poor prognosis. The prevalence of anaemia in CHF like in SOLVD trial 22% and appears to increase with worsening of NYHA class. Worsening anaemia can contribute to deterioration of CHF.Theetiology of anaemia in CHF is multi-factorial- from poor dietary intake, blood loss consequent to co-administered anti-platelet drugs and poor utilisation of iron due to elevated cytokines, TGF, IL-6 and markedly elevated hepcidin. This patient had rare type of anaemia with markedly elevated serum folic acid(>2000ng/ml) and normal serum vitamin B12 levels. This markedly elevated serum folic acid levels occurs in stagnant bowel loop (blind loop) syndrome, where gut bacteria produce and contribute to markedly elevated folic acid level. This finding is indicative of associated gut disease either stricture, blind loop- in patients with CHF.

12 2D ECHO 2D echo with Doppler cannot only confirm the clinical suspicion of systolic heart failure, by low EF, but also etiology-valvular pathology, pericardiac disease, endocardiac and myocardial disorder. It is a good imaging technology, where thickness of muscle and cavity size, and myocardial texture, in addition to hemodynamic data cardiac output,filling pressuresis available. Disproportionate RV/RA dilation and dysfunction in patients with left heart disease should raise suspicious of associated pulmonary embolism like in this patient. Incidence of PTE in CHF is approximately twice that of non CHF patient are mainly due to immobility and venous stasis (due to low cardiac output). This patient has PTE with pulmonary infarct (as in HRCT), also aggravated arterial hypoxia(spo2: 90%). Generally subsegmental PTE has no significant impact in patients without any underlying CV disease. But in patients who has underlying heart disease can cause hemodynamic perturbation.this could have been prevented by prophylactic heparin(ufh in patients with CKD)

13 This patient echo is characterized by Global Hypokinesia (LVEF 33.6%) with normal myocardial thickness. PH(RVSP=58) with severe TR and RV dysfunction (TAPSE 1.7). Cardiac valves and pericardium were reported to be normal. The occurrence of PH in patients with cardiomyopathy is approximately 60%. The prevalence and impact of PH on prognosis seem to depend on type of myopathy, with worse impact (higher mortality) in patients with myocarditis than other type of cardiomyopathy.

14 Should we investigate all patients, for potential salvageable patients like myocarditis or diseases? If so what is the approach to etiologic diagnosis? The answer is yes and it involves three staged diagnostic process (ESC-2015guidlines) Stage I- (SCREENING) Stage II- Identification of diagnosable causes Stage III- Establishing etiology of Idiopathic dilated causes to differentiate between two main causes of Idiopathic DCM- 1) Genetic 2) Inflammation/autoimmune

15 Symptomatic heart failure (HF) + left ventricular systolic dysfunction (LVSD) Exlusion Of CAD -CAG echocardiographyprimary heart valve disease & Congestive heart Disease Arterial hypertension history + additional tests Dialated cardiopathy(dcm) exclusion of other types of cardiomyopathy medications- induced cardiomyopathy connective tissue diseases tachycardia induced cardiomyopathy endocrinologic disorders infiltrative diseases toxins induced cardiomyopathy other miscellaneous causes Idiopathic dilated cardiomyopathy (IDCM)

16 This patient with Diabetes Mellitus had multiorgan involvement- HEART, Lung, Intestine which makes one consider granulomatous carditis as a distinct possibility like sarcoidosis and Tuberculosis. The possibility of Tuberculosis is high due to following reasons: Patient is Diabetic HRCT- of chest at Basal slice is suggestive of tree in bud appearance which characterises endobronchial spread of infection. The slice at subcarinal level shows mass probably lymph nodes. No data is available regarding further pursuance of mass Biopsy, PET scan etc.

17 Myocardial involvement by mycobacteria before era of chemotherapy has been reported in range of 0.3% at autopsy, most commonly pericardium and rarely myocardium.three different types of myocardial involvement has been described- Diffuse infiltrative miliary and nodular (Tuberculoma). Extra pulmonary site is seen in only 25% cases. The best imaging modality for diagnosis of diffuse infiltrative type (likely to cause LV systolic Dysfunction) MRI imaging with short T1 inversion recovery. The image shows edema of myocardium and fibrosis of epicardium. The spread of disease is either by haematogenous route or by contiguous from adjacent lymph nodes, and in some by nirtograde spread via lymphatic presenting symptoms include systolic ventricular dysfunction (LVF) or ventricular dysarrhythmias.

18 Immediate cause of death Arrhythmias : LV systolic dysfunction with EF <35%. Recurrence of PTE inspite of UFH therapeutic failure rate - approximately 3.5% FINAL DIAGNOSIS: LV Systolic Dysfunction probably due to granulomatous myocarditis. Complicated by PTE sub segmental with pulmonary infarct Diabetes Mellitus Stagnant loop syndrome.

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