Cardiomyopathy diastolic dysfunction

Heart failure (HP) may becausedbya primary abnormality in systolic function, diastolic function, or both. Making the distinction is important because the prevalence, prognosis, and treatment of HP may be quite different depending on whether the predominant mechanism causing the symptoms is systolic or diastolic dysfunction. Some clinical studies have reported that as many as 30% to 50% of patients with congestive heart failure have preserved left ventricular (LV) function, making diastolic heart failure (DHP) very common. In addition, abnormalities in diastolic function also can play an important role in the development of symptoms in patients with cardiomyopathy and systolic heart failure (SHP). 

In dilated cardiomyopathy, the cardinal feature is dilatation of the ventricles. Systohc fnnction is abnormal, leading to a decreased cardiac ontpnt. Inpatients with hypertrophic cardiomyopathy (HCM), the ventricnlar cavity is not dilated, and the ventricnlar mnscle mass is increased. Ventricnlar cavity size is normal or decreased, and systolic function often is preserved. Patients with HCM may have an obstructive or nonobstructive form. Patients with restrictive cardiomyopathy have inadequate ventricular comphance causing diastolic dysfunction owing to endocardial and/or myocardial disease. The chnical presentation is similar to that of constrictive pericarditis. 

Udelson JE, Bonow RO. Left ventricular diastolic function and calcium channel blockers in hypertrophic cardiomyopathy. In Gaasch WH, Le Winter MM, eds. Left Ventricular Diastolic Dysfunction and Heart Failure. Philadelphia, Lea Febiger, 1994 465. 

Systolic dysfunction, or decreased contractility, can be caused by dilated cardiomyopathies, ventricular hypertrophy, or a reduction in muscle mass. Diastolic dysfunction, or restriction in ventricular filling, can be caused by increased ventricular stiffness, mitral or tricuspid valve stenosis, or pericardial disease. Both ventricular hypertrophy and myocardial ischemia can contribute to increased ventricular stiffness. Angiotensin II causes and/or exacerbates heart failure by increasing systemic vascular resistance, promoting sodium retention. 

The literature includes eight case reports of pulmonary hypertension in AL amyloidosis attributed to pulmonary vascular amyloid deposition (Table 2). Autopsies confirmed pulmonary artery amyloid deposits in four out of four cases. The prevalence of restrictive cardiomyopathy and diastolic dysfunction in AL patients predisposes them to secondary forms of pulmonary vascular disease. Direct measurements of pulmonary artery pressures (PAP) were obtained in five cases (3 pulmonary arteriograms, 2 right heart catheterizations) however, only two reports include direct measures of left atrial filling pressures. Echocardio-graphic estimates of elevated right ventricular systolic pressures and normal diastolic function were reported in all cases. 

The retrospective analysis of 35 cases of large persistent AL pleural effusions versus 120 AL cardiomyopathy patients without effusions offers perspective on the role of cardiac dysfunction and elevated filling pressures on amyloid pleural effusions. No differences in multiple echocardiographic parameters (interventricular septal thickness, left ventricular ejection fraction, or sensitive measures of diastolic function) could be identified between the pleural effusions group and the effusion-free cardiomyopathy group (21). Ironically, nephrotic range proteinuria and hypoalbuminemia were more prevalent in the... 

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