TABLE 2.
Cause | Clinical presentation | Differentiating features |
---|---|---|
Secondary to LV failure | Typical heart failure presentation | Abnormal LV valves with evidence of increased filling pressures |
Hypoxia in advanced stages | Can confirm via left heart or transeptal catheterization | |
High BNP when decompensated | ||
Secondary to PAH | RHF | Evidence of pulmonary hypertension |
Hypoxia may occur earlier | No evidence of increased LV filling pressures | |
Evidence of significant lung disease may be present | May require cardiac catheterization to determine LV filling pressures | |
Findings of pulmonary hypertension may be present | BNP may be modestly elevated | |
Clinical findings may reflect the presence of conditions associated with PAH such as scleroderma | ||
Secondary to RV myopathic process | RHF | Diagnosis can usually be made on clinical grounds and with echocardiography or CMR |
RV infarction | Acute or post-MI presentation | May need urgent right heart catheterization to determine RV and LV filling pressures |
May also have LV failure | Low cardiac output despite elevated JVP following acute MI | |
May not tolerate vasodilator therapy due to systemic hypotension | ||
ARVC | Familial, uncommon (10%) LV involvement, may be asymptomatic | – |
Other rare cardiomyopathy* | Variable | – |
Restrictive cardiomyopathy | RHF | Pulmonary hypertension may be present |
May mimic constriction | BNP may be very high | |
Mixed RV/LV failure | ||
Pericardial disease† | RHF without evidence of pulmonary hypertension | Pulmonary hypertension absent |
May see abnormal pericardium | ||
May differentiate from restrictive cardiomyopathy by tissue Doppler assessment | ||
Cardiac catheterization and/or RV biopsy may be required for differentiation | ||
Right-sided valvular heart disease | Clinical findings of pulmonary or tricuspid valve disease | Evidence of severe valvular structural and functional abnormality |
Usually observed by echocardiography | ||
Associated condition present (eg, endocarditis, carcinoid, diet pill ingestion) | Evidence of interference of tricuspid closure by pacing wire, long history of RV pacing, with no other cause for ventricular dysfunction | |
History of RV pacing | ||
Congenital heart disease | Highly variable but, frequently, a history of congenital heart disease precedes RHF presentation | Congenital heart disease noted by echocardiography or CMR |
Unexplained increase in RV volume warrants careful evaluation to rule out atrial septal defect or other intracardiac shunt; transesophageal echocardiography may be necessary |
Uhl’s anomaly, Chagas’ disease (uncommon in North America, common elsewhere), right-sided involvement of hypertrophic cardiomyopathy;
Mimic of RHF. ARVC Arrhythmogenic right ventricular cardiomyopathy; BNP B-type natriuretic peptide; CMR Cardiac magnetic resonance imaging; JVP Jugular venous pressure; LV Left ventricle; RV Right ventricle; MI Myocardial infarction; PAH Pulmonary arterial hypertension