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Restrictive cardiomyopathy is the least common type of cardiomyopathy, being defined by diastolic dysfunction and often unimpaired systolic function. Restrictive cardiomyopathies can be classified as familial or non-familial. Patients with familial restrictive cardiomyopathy can develop signs and symptoms of this condition anytime from childhood to adulthood. The evolution of the disease is towards signs and symptoms of pulmonary and systemic congestion and, without treatment, there is a fiveyear mortality rate of approximately 30% in these patients. We discuss the case of a 43-year-old patient diagnosed with familial restrictive cardiomyopathy with positive genetic tests for mutations of MYH7 gene and ABCC9 gene, who was first hospitalized in 2011 for palpitations. The echocardiography performed in evolution showed a continuous alteration of right ventricle function, without important differences of left ventricular function. She developed heart failure symptoms six years after diagnosis and she had seven hospitalizations in the past two years, currently with an increasing need of diuretics and persistent hepatic dysfunction. Cardiac transplantation or left ventricular assist device therapy should be considered in patients with severe heart failure symptoms and no longer effective treatment. However, elevated pulmonary vascular resistance excludes the patient from cardiac transplantation.

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Objectives. To evaluate the left ventricular diastolic function in patients with cirrhosis and to establish its relation to the degree of severity of liver disease as well as to biological markers of cardiac dysfunction. Methods. We included 72 patients diagnosed with liver cirrhosis in different degrees of disease progression. Cardiac parameters were evaluated by two-dimensional echocardiography and color Doppler. Results. In terms of diastolic echocardiographic function, 26 patients had normal diastolic function, 36 had grade 1 diastolic dysfunction, 8 had grade 2 dysfunction, and 2 patients had grade 3. Diastolic dysfunction was unrelated to age, gender and etiology of cirrhosis. Of all the echocardiographic parameters, left ventricular hypertrophy, right cavity dimensions, E wave deceleration time, left atrial volume were significantly higher in patients with diastolic dysfunction, correlated with the severity of cirrhosis. Significantly elevated NT-proBNP and TnI were seen among the patients with diastolic dysfunction. Conclusions. The present study shows that although diastolic dysfunction is common in cirrhosis, it is usually incipient and most often correlates with the severity of the liver disease (Child-Pugh class).

Acute pulmonary edema is an often encountered medical emergency characterized by sever shortness of breath, orthopnea and pulmonary overload caused by left ventricular failure. The specific pharmacological and nonpharmacological therapies should be administered rapidly and their objective is to correct the hypoxemia and reduce the left ventricular afterload. Although the symptoms often regress quickly, the in-hospital and short-term mortality remain high. To improve the prognosis of these patients, long-term management is as important as the one in the acute setting, and includes the patient education, the correct and complete medical treatment of the subjacent heart disease and the prevention of risk factors.