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A previously healthy young woman with a rapidly developed dyspnoea and thick-walled left ventricle

The patient is a 25-year old previously healthy woman who had studied building branch and worked at construction sites until March 2009 when she became unemployed. Her only regular medication comprised contraceptive pills (desogestrel and ethinylestradiol). She had been thin all her life (169,5cm/ 49 kg, BMI 17.1 kg/m2) with a blood pressure of 105/50 mmHg in February 2009. She did not smoke or use drugs neither had alcohol abuse. Her mother had rheumatoid artrhitis and her father had died accidentally. Neither of them was diagnosed with any cardiac disease. The patient was the only child.

In September 2009 she had initially some fever, then prolonged cough and symptoms of a common cold. Sinuitis was suspected whereafter antibiotics and a combination drug containing ASA 350mg per tablet (which she took up to four tablets a day) were prescribed. However, she had to be admitted to the hospital in November 2009 due to progressive dyspnoea. On the previous day, her blood pressure was 120/65 mmHg and pulse rate 110/min. She reported also some difficulties in swallowing and before admission to the hospital she had inadvertently lost weight about four kilograms.

At the hospital emergency her respiratory frequency was 29/min, oxygen saturation 80%, blood pressure was 245/157mmHg and she had sinus tachycardia 107/min. Blood gas analyses demonstrated metabolic alcalosis. Serum creatinine concentration was increased (216 umol/l, normal range 50-90 umol/l). There was hematuria, mild proteinuria, hyponatremia (117 mmol/l, normal range 137-145 mmol/l) and mild anemia. Blood thrombocyte count was normal. Plasma potassium concentration was repeatedly normal. Crp was 7 mg/l and erythrocyte sedimentation rate 13 mm/h. The concentration of plasma natriuretic peptide was high (34 568 ng/l, normal range <155 ng/l) whereas the concentrations of plasma CK, CKMBm and lactate were normal. ECG showed LVH


Fig.1: The first ECG showed sinustachycardia, LVH and negative p-waves in V1, 
          suggestive of left atrial abnormality

According to hospital records, in initial bed-side echocardiography the maximal left ventricular wall thickness measured up to 18 mm. In addition the left ventricular contractility was diminished (Fig. 2).


Fig.2: The main findings of the bedside echocardiography at the emergency were
          thickened left ventricular walls and diminished left ventricular contractility (EF 36%).

The E/A relation was inversed and there were mild aortic and mitral valve regurgitations (not shown). Thorax X-ray demonstrated enlarged heart, pulmonary oedema and moderate bilateral pleural effusions. Medical treatment for hypertension and alveolar oedema was promptly initiated.


1. What is your working hypothesis and differential diagnoses?
2. Which further evaluations would you suggest?


Diagnosis and further management

The main initial observations were hypertensive crisis, alveolar oedema, renal failure and acute heart failure.  Echocardiography showed thickened left ventricular walls, systolic and diastolic dysfunction.

The reason for extremely high blood pressure was sought. The patient did not use drugs. She used to have every now and then some salty liquorice but not every day. Contraception pills could have caused hypertension but usually it would have been milder. The possibilities of primary hyperaldosteronism, acromegaly, Liddle’s syndrome, Cushing’s disease, pheochromocytoma, thyroid dysfunction, primary hyperparathyreosis or undiagnosed coarctation of the aorta were excluded.

The possibility that both left ventricular hypertrophy and renal failure could have been caused by a storage disease or by amyloidosis, was discussed. In further evaluation, there was no evidence of a monoclonal gammopathy.

The ethiology of renal failure was examined further. It remained unclear, whether it was acute or chronic. The findings in the renal ultrasound were nonremarkable. Renal MRI did not show any  abnormalities and angiography demonstrated normal renal arteries, no signs of fibromuscular dysplasia or atherosclerosis. Renal biopsy done in November 2009 was representative with 22 glomeruli. There were no signs of a glomerulonephritis. Immunofluorescence stainings were negative in glomeruli. There was no amyloid. The  main finding was significant, concentric, onion-like obliteration of small arterioles with hyalin deposits and fibrinoid necrosis. The biopsy findings were compatible with malignant hypertension, systemic scleroderma, haemolytic uremic syndrome or thrombocytopenic thrombotic purpura (TTP). The patient did not have findings diagnostic for scleroderma. The difficulty in swallowing passed by and the results of the oesophageal manometry were normal. The possibility of TTP was excluded by repeatedly normal thrombocyte count. The patient did not have coeliac disease or SLE not to speak about epidemic nephropathy.

It was concluded that the process was probably initiated by malignant hypertension. This view was supported by the fact that the proteinuria disappeared as normotension was achieved with enalapril, bisoprolol and amlodipin. Renal failure may have possibly been worsened by the use of ASA.  ECG (Fig.3) left ventricular systolic function (Fig. 4), mitral inflow pattern and Em/Am of the mitral valve lateral annulus (not shown) had normalized by February 2010. Since the cardiac abnormalities were interpreted to be secondary to renal disease,EMB was not  considered.


As the ECG taken in August 2010 is compared to those taken in November 2009, a remarkable resolution of the LVH changes can be observed.

The left ventricular wall thickness and systolic function had improved rapidly as demonstrated by the echocardiography carried out in February 2010.



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Notes to editor

By Dr. Tiina Heliö (1) and Dr. Sari Aaltonen (2),
(1) Helsinki University Central Hospital, Department of Medicine, Division of Cardiology, Helsinki, Finland
(2) Helsinki University Central Hospital, Department of Medicine, Division of Nephrology, Helsinki, Finland

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.