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Circulation. 2022;145:e895–e1032. DOI: 10.1161/CIR.0000000000001063 May 3, 2022 e909 CLINICAL STATEMENTS AND GUIDELINES

distention,17 orthopnea,18 bendopnea,19 a squarewave response to the Valsalva maneuver,20 and leg edema.6 On a practical level, clinicians use extent of clinical congestion to guide titration of pharmacological treatments, including doses of diuretics. Observational studies have shown that clinical congestion is an important adverse risk factor in patients with HF.1–6,17 Recently, the PARADIGM-HF (The Efficacy and Safety of LCZ696 Compared to Enalapril on Morbidity and Mortality of Patients With Chronic Heart Failure) investigators showed that, in patients with chronic HFrEF, changes in markers of clinical congestion were associated with QOL as assessed by the Kansas City Cardiomyopathy Questionnaire and also provided prognostic information independently even of natriuretic peptides or the MAGGIC (Meta-analysis Global Group in Chronic Heart Failure) risk score.2 These data highlight the ongoing relevance of clinical congestion ascertained by the history and physical examination. 2.	 Some patients with HF progress to an advanced state, a condition that can be treated with specialized interventions such as mechanical circulatory support (MCS) or cardiac transplantation. Such patients should be identified before they progress to a state of extremis, at which point they may succumb to their illness or suffer complications of an intervention as a result of their very advanced state. Several “simple clinical clues” are available to identify advanced HF and should be ascertained via a focused history and physical examination. The recognition that a patient has advanced HF will allow for earlier referral to an advanced HF center, when appropriate, as will be discussed later in this document (see Section 8, “Specialty Referral for Advanced HF”). 3.	 Increasingly, familial cardiomyopathy is recognized as a more accurate diagnosis in some patients previously classified as having an idiopathic dilated cardiomyopathy (DCM). A detailed family history may provide the first clue of a genetic basis. A broad array of questions includes whether family members had a weak, enlarged, or thick heart, or HF; muscular dystrophy; a pacemaker or defibrillator; were on a heart transplant list; or died unexpectedly. Periodic updating of the family history in patients with a cardiomyopathy of uncertain origin may lead to a diagnosis of familial cardiomyopathy in the event that a relative subsequently develops a cardiomyopathy or a related complication. A 3-generation family pedigree obtained by genetic health care professionals improved the rate of detection of a familial process as compared with routine care.14 Furthermore, a family history of cardiomyopathy, as determined by a 3-generation pedigree analysis, was associated with findings of gadolinium enhancement on cardiac magnetic resonance imaging (MRI) and increased major adverse cardiac events.13 The possibility of an inherited cardiomyopathy provides the impetus for cascade screening of undiagnosed family members, thereby potentially avoiding preventable adverse events in affected relatives by implementation of GDMT and other management that otherwise would not be initiated. 4.	 Certain conditions that cause HF require diseasespecific therapies. For example, in amyloid heart disease, whether on the basis of transthyretin21 or light chain deposition,22 there are specific treatments that otherwise would not be used in patients with HF. Hence, expeditious and accurate diagnosis of such conditions is important. Currently, important delays have been reported in diagnosing amyloid heart disease,16 perhaps not unexpectedly given the wide spectrum of possible clinical presentations.15 Similarly, HF attributable to sarcoidosis, hemochromatosis, hypothyroidism, hyperthyroidism, acromegaly, connective tissue disease, tachycardia-induced cardiomyopathy, or highoutput HF from an arteriovenous fistula, among others, requires specific therapeutic approaches. Given that the differential diagnosis of HF is broad, the history and physical examination can provide clues to narrow the number of causes to consider and guide the diagnostic approach to identify such conditions (Table 5). 5.	 The history and physical examination help to identify the cause of a clinical deterioration. To determine the cause of a clinical deterioration, the clinician assesses for concurrent illness (eg, ongoing myocardial ischemia, pulmonary emboli, or systemic infection), initiation of a medication potentially detrimental in the setting of HF (eg, nonsteroidal anti-inflammatory drugs [NSAIDs]), or the possibility of chronic RV pacing (eg, a newly implanted pacemaker or medications such as amiodarone that leads to bradycardia and resultant chronic RV pacing), nonadherence to a medication or dietary regimen, and ongoing substance abuse. In addition, an assessment of social determinants of health (eg, housing stability, food security, available transportation) should be made.

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