Case report on rare case of dilated cardiomyopathy in young girl
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0975-2870.157096
Takayasu's arteritis is a large vessel vasculitis in which the inflammatory process involves aorta and major branches. The cause is largely unknown. Dilated cardiomyopathy (DCM) is, however, reported to be seen in only 5-6% of cases of Takayasu arteritis. We report a rare case of DCM with renovascular hypertension secondary to Takayasu's arteritis. Keywords: Dilated cardiomyopathy, renal artery hypertension, Takayasu′s arteritis
Dilated cardiomyopathy (DCM) is one of the cardiomyopathies, a group of diseases that primarily affect the myocardium (the muscle of the heart). [1] Different cardiomyopathies have different causes and affect the heart in different ways. In DCM, the heart becomes weakened and enlarged and cannot pump blood efficiently. The decreased heart function can affect the lungs, liver, and other body systems. [1] In DCM, a portion of the myocardium is dilated, often without any obvious cause. Left ventricular (LV) or right ventricular systolic pump function of the heart is impaired, leading to progressive cardiac enlargement and hypertrophy, a process called remodeling. [1] About one in three cases of congestive heart failure (CHF) is due to DCM. [1] About 25-35% of patients have familial forms of the disease, [1] with most mutations affecting genes encoding cytoskeletal proteins, [2] while some affect other proteins involved in contraction. [2] Takayasu's arteritis (also known as "aortic arch syndrome," "nonspecific aortoarteritis," and the "pulseless disease") is a form of large vessel granulomatous vasculitis [1] which causes massive intimal fibrosis and narrowing of arteries, affecting often young or middle-aged women of Asian descent. It mainly affects the aorta and its branches, as well as the pulmonary arteries. Females are 8-9 times more affected than males. [1],[3] Patients often notice the disease symptoms between 15 and 30 years of age. Takayasu's arteritis is similar to other forms of vasculitis, including giant cell arteritis. [3],[4] Due to obstruction of the main branches of the aorta, including the left common carotid artery, the brachiocephalic artery, and the left subclavian artery, Takayasu's arteritis can present as pulseless upper extremities (arms, hands, and wrists with weak or absent pulses on the physical examination), commonly referred to as the "pulseless disease" and renal arteries are involved. In India, Takayasu arteritis as cause of renovascular hypertension is reported in 28-75%, CHF in 76%, aortic regurgitation in 20-24% [5] and DCM is, however, reported to be seen in only 5-6% of cases of Takayasu arteritis. [4] The presentation as DCM is rarely reported and is due to involvement of coronary artery, severe hypertension, and cardiac failure are bad prognostic factors. [1],[2]
A 14-year-old female presented with complaints of breathlessness on exertion since 6 months and aggravated since 2 days prior to admission, sudden in onset, New York Heart Association classification Grade 3, aggravated on lying down and relieved at rest. Patient also complained of cough since 2 days prior to admission, associated with expectoration, whitish in color associated with complains of chest pain, retrosternal in location aggravated on coughing. There was no history of fever/palpitations. On general physical examination, pulse rate was 102 beats/min of left radial artery, regular in rate, rhythm. Right upper limb arterial pulsations were absent. There was no radio-femoral delay. Blood pressure was 160/100 mmHg of left arm in the supine position. Moderate pallor and facial puffiness were present. Bilateral pedal edema was present which was of pitting type. Jugular venous pressure was raised. On examination, cardiovascular system revealed apex beat was shifted downward and outward. Heart sounds were normal, tachycardia was present. On respiratory system examination, bilateral basal crepitations were present. Per-abdomen examination suggestive of hepatomegaly, liver span-15 cm. On central nervous system, examination patient was conscious and oriented. Investigations Hemogram revealed hemoglobin of 10.4 g%, total leucocyte count was 7000 cells/cumm, platelet count was 2.8 lakhs/cumm. Erythrocyte sedimentation rate was 56 mm at the end of 1 st h. C-reactive protein was positive. Renal function tests were normal. Blood urea was 44 mg%, and serum creatinine was 0.9 mg%. Serum electrolytes were normal. Serum sodium was 136 mmol/L, serum potassium was 4.3 mmol/L, serum calcium was 9.4 mg%, serum phosphorus was 3.4 mg%. Anti-nuclear antibodies were negative, and anti-ds-DNA was negative. Electrocardiography showed sinus tachycardia with LV hypertrophy with left bundle branch block (LBBB) chest X-ray was suggestive of cardiomegaly [Figure 1] and [Figure 2]. Two-dimensional echo revealed DCM, global LV hypokinesia, severely depressed LV systolic function LV-ejection fraction-30% [Figure 3]. Color arterial Doppler of right upper extremity showed monophasic flow noted in infraclavicular subclavian artery with narrowing of right subclavian artery. Monophasic flow noted distally in axillary, brachial, radial, and ulnar artery. The peak flow velocities are reduced. Findings are suggestive of obstruction in the aortic arch region.
Whole body positron emission tomography-computed tomography report revealed Significant narrowing of right subclavian artery in its entire course and wall-thickening causing significant narrowing of descending thoracic aorta seen [Figure 4]. No significant metabolic activity seen at these sites to suggest any active inflammation. Mildly fludeoxyglucose avid supraclavicular and mediastinal lymph nodes? Residual inflammatory nodes.
Computed tomography-angiography of aorta showed Significant narrowing of right subclavian artery in its entire course with high-grade stenosis at the origin of the right vertebral artery.
Renal Doppler showed that the abdominal aorta showed irregular wall-thickening, especially in renal and suprarenal portion. Maximum diameter was 8 mm. The renal arteries were seen originating from this narrowed portion. The renal arteries on either side at origin, hilum and intra-renal portion show low systolic velocities with significant spectral widening. These changes are secondary to aorta flow and likely to be a spectrum of the same disease. The inferior vena cava was thrombus free. The renal veins were normal. Patient was treated with steroids, diuretics and anti-hypertensives, antibiotics. Patient got discharged and was maintained on follow-up therapy with the same. On second hospital admission, patient came with same complaints as before and admitted with high blood pressure and was in congestive cardiac failure New York Heart Association classification Grade 4 and was treated for the same with diuretics, anti-hypertensives like calcium channel blockers, prazosin, steroids, anti-platelets, carvedilol, and tablet digoxin. Hemogram showed hemoglobin of 11.4 g%, total leucocyte count was 9000 cells/cumm platelet count was 2.5 lakhs/cumm, erythrocyte sedimentation rate was 34 mm at end of 1 st h. Renal function tests were normal. Blood urea was 42 mg%, serum creatinine was 0.8 mg%. Serum electrolytes revealed serum sodium was 138 mmol/L, serum potassium was 4 mmol/L, serum calcium was 8.4 mg%, serum phosphorus was 3.6 mg Electrocardiography showed sinus tachycardia with LV hypertrophy with LBBB. Chest X-ray was suggestive of cardiomegaly. Two-dimensional echo showed DCM, global LV hypokinesia, severely depressed LV systolic function LV-ejection fraction-30%. Four days after admission, CHF reduced, and patient had two episodes of seizures. Computed tomography-brain showed small gliotic area seen in body of right caudate nucleus mostly sequelae to old ischemic infarct. Patient was treated with anti-epileptics-injection phenytoin sodium. Next day, patient complained of breathlessness at rest, sudden in onset associated with complains of cough with expectoration. Chest X-ray was suggestive of cardiomegaly with pulmonary edema which progressed to acute respiratory distress syndrome, patient was intubated for the same. Fifteen minutes after intubation, patient developed asystole. Cardiorespiratory measures were given, but patient could not be revived.
Takayasu arteritis is the most common cause of renovascular hypertension in India. Indian origin aortoarteritis is a chronic granulomatous, necrotizing vasculitis, predominantly affecting the aorta with its branches. [6] Though the exact pathogenesis of the arteritis is still unknown, tuberculosis, streptococcal infections, rheumatoid arthritis, and other collagen vascular diseases have been debated as its etiology in the past. Recently, more emphasis has been given on an immunopathological cause. [6],[7] The disease is classified based on the site of involvement: [8]
The site of arterial disease determines its clinical presentation. In our patient, there is involvement of descending thoracic aorta, right subclavian artery, and renal arteries are involved. Type III-Takayasu's arteritis. The presentation as DCM is rarely reported and is due to involvement of coronary artery, severe hypertension, and cardiac failure are bad prognostic factors. [9],[10] The progression of heart failure is associated with LV remodeling, which manifests as gradual increases in LV end-diastolic and end-systolic volumes, wall thinning, and a change in chamber geometry to a more spherical, less elongated shape. This process is usually associated with a continuous decline in ejection fraction. [11] Death is due to either CHF or ventricular tachy-or bradyarrhythmias. In our patient, death is due to acute respiratory distress syndrome due to congestive cardiac failure secondary to DCM and renal vascular hypertension secondary to Takayasu arteritis. Therapeutic modalities include steroids, immunosuppressive agents, and antihypertensive drug therapy. 20-100% success rate of steroids have been reported in different studies. [12] Cyclophosphamide and methotrexate are often needed to control intense inflammatory response. In addition, balloon dilatation or stenting is often necessary. [13] Drug therapy can slow down progression of cardiomyopathy and in some cases even improve the heart condition. Standard therapy may include salt-restricted diet, diuretics, and digitalis. [1]
With this case report, we want to emphasize that young female patient with hypertension, heart failure, and/or renal failure should be screened for systemic vasculitis, since only an anti-inflammatory approach can avoid or at least reduce other complication of vasculitis.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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