Table 1

Summary of specific heart failure with preserved ejection fraction differential diagnosis and relative treatment

ConditionClinical and instrumental findingsTherapy
Hypertrophic cardiomyopathy (HCM)Asymmetric or global increase in LV wall thicknessGeneral: avoid dehydration, rhythm control if AF
Possible LVOT obstructionObstructive HCM: BB, CCB, disopyramide, mavacamten, SRT49
Non-obstructive HCM: valsartan may slow LV remodelling when administered early50
Cardiac amyloidosisGlobal increase in LV wall thicknessTTR: tafamidis and other novel treatments51
Restrictive pathophysiologyAL: haematological treatment
Pulmonary hypertension (PH) not due to left-sided HF (Groups 1, 3, 4, and 5)Type 1: idiopathic/hereditary PHAetiology-based treatment
Type 3: pulmonary disease–related PHSpecialist referral
Type 4: CTPH
Type 5: rare disease
High-output HFElevated cardiac output may be due to obesity, arteriovenous shunts, liver disease, lung disease, and myeloproliferative diseaseAetiology-based treatment
Fabry diseaseX-linked genetic transmissionERT
Systemic symptoms/renal involvement
Restrictive pathophysiology
Cardiac sarcoidosisUsually related to extracardiac sarcoidosisImmunosoppression
Possible symptoms include ventricular arrythmias and heart failureSpecialist referral
Constrictive pericarditisRelated to chest surgery or trauma, history of pericarditis, or connective tissue diseaseAnti-inflammatory treatment if active pericarditis
Pericardiectomy
ConditionClinical and instrumental findingsTherapy
Hypertrophic cardiomyopathy (HCM)Asymmetric or global increase in LV wall thicknessGeneral: avoid dehydration, rhythm control if AF
Possible LVOT obstructionObstructive HCM: BB, CCB, disopyramide, mavacamten, SRT49
Non-obstructive HCM: valsartan may slow LV remodelling when administered early50
Cardiac amyloidosisGlobal increase in LV wall thicknessTTR: tafamidis and other novel treatments51
Restrictive pathophysiologyAL: haematological treatment
Pulmonary hypertension (PH) not due to left-sided HF (Groups 1, 3, 4, and 5)Type 1: idiopathic/hereditary PHAetiology-based treatment
Type 3: pulmonary disease–related PHSpecialist referral
Type 4: CTPH
Type 5: rare disease
High-output HFElevated cardiac output may be due to obesity, arteriovenous shunts, liver disease, lung disease, and myeloproliferative diseaseAetiology-based treatment
Fabry diseaseX-linked genetic transmissionERT
Systemic symptoms/renal involvement
Restrictive pathophysiology
Cardiac sarcoidosisUsually related to extracardiac sarcoidosisImmunosoppression
Possible symptoms include ventricular arrythmias and heart failureSpecialist referral
Constrictive pericarditisRelated to chest surgery or trauma, history of pericarditis, or connective tissue diseaseAnti-inflammatory treatment if active pericarditis
Pericardiectomy

AF, atrial fibrillation; AL, amyloid light chain; BB, beta blockers; CCB, calcium channel blockers; CTPH, chronic thromboembolic pulmonary hypertension; ERT, enzyme replacement therapy; LV, left ventricular; LVOT, left ventricular outflow tract; SRT, septal reduction therapy; TTR, transthyretin.

Table 1

Summary of specific heart failure with preserved ejection fraction differential diagnosis and relative treatment

ConditionClinical and instrumental findingsTherapy
Hypertrophic cardiomyopathy (HCM)Asymmetric or global increase in LV wall thicknessGeneral: avoid dehydration, rhythm control if AF
Possible LVOT obstructionObstructive HCM: BB, CCB, disopyramide, mavacamten, SRT49
Non-obstructive HCM: valsartan may slow LV remodelling when administered early50
Cardiac amyloidosisGlobal increase in LV wall thicknessTTR: tafamidis and other novel treatments51
Restrictive pathophysiologyAL: haematological treatment
Pulmonary hypertension (PH) not due to left-sided HF (Groups 1, 3, 4, and 5)Type 1: idiopathic/hereditary PHAetiology-based treatment
Type 3: pulmonary disease–related PHSpecialist referral
Type 4: CTPH
Type 5: rare disease
High-output HFElevated cardiac output may be due to obesity, arteriovenous shunts, liver disease, lung disease, and myeloproliferative diseaseAetiology-based treatment
Fabry diseaseX-linked genetic transmissionERT
Systemic symptoms/renal involvement
Restrictive pathophysiology
Cardiac sarcoidosisUsually related to extracardiac sarcoidosisImmunosoppression
Possible symptoms include ventricular arrythmias and heart failureSpecialist referral
Constrictive pericarditisRelated to chest surgery or trauma, history of pericarditis, or connective tissue diseaseAnti-inflammatory treatment if active pericarditis
Pericardiectomy
ConditionClinical and instrumental findingsTherapy
Hypertrophic cardiomyopathy (HCM)Asymmetric or global increase in LV wall thicknessGeneral: avoid dehydration, rhythm control if AF
Possible LVOT obstructionObstructive HCM: BB, CCB, disopyramide, mavacamten, SRT49
Non-obstructive HCM: valsartan may slow LV remodelling when administered early50
Cardiac amyloidosisGlobal increase in LV wall thicknessTTR: tafamidis and other novel treatments51
Restrictive pathophysiologyAL: haematological treatment
Pulmonary hypertension (PH) not due to left-sided HF (Groups 1, 3, 4, and 5)Type 1: idiopathic/hereditary PHAetiology-based treatment
Type 3: pulmonary disease–related PHSpecialist referral
Type 4: CTPH
Type 5: rare disease
High-output HFElevated cardiac output may be due to obesity, arteriovenous shunts, liver disease, lung disease, and myeloproliferative diseaseAetiology-based treatment
Fabry diseaseX-linked genetic transmissionERT
Systemic symptoms/renal involvement
Restrictive pathophysiology
Cardiac sarcoidosisUsually related to extracardiac sarcoidosisImmunosoppression
Possible symptoms include ventricular arrythmias and heart failureSpecialist referral
Constrictive pericarditisRelated to chest surgery or trauma, history of pericarditis, or connective tissue diseaseAnti-inflammatory treatment if active pericarditis
Pericardiectomy

AF, atrial fibrillation; AL, amyloid light chain; BB, beta blockers; CCB, calcium channel blockers; CTPH, chronic thromboembolic pulmonary hypertension; ERT, enzyme replacement therapy; LV, left ventricular; LVOT, left ventricular outflow tract; SRT, septal reduction therapy; TTR, transthyretin.

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