Is it consistent for a patient with Hypertrophic Cardiomyopathy (HCM), constrictive pericarditis, and diastolic dysfunction to present with a normal Ejection Fraction (EF), low Cardiac Output (CO), mild Left Ventricular Outflow Tract (LVOT) obstruction, steep velocity of propagation of mitral inflow, high E/e' ratio, and low e' velocities?

From the Guidelines

Yes, this presentation is consistent with a patient who has Hypertrophic Cardiomyopathy (HCM), constrictive pericarditis, and diastolic dysfunction. The normal ejection fraction with low cardiac output is a classic finding in these conditions, particularly when diastolic dysfunction is present, as noted in the 2024 guidelines for the management of HCM 1. In these disorders, the heart's ability to relax and fill properly during diastole is impaired, while systolic function (measured by EF) often remains preserved. The mild LVOT obstruction is typical in HCM due to septal hypertrophy, and its dynamic nature is influenced by loading conditions, as discussed in the 2024 AHA/ACC/AMSSM/HRS/PACES/SCMR guideline for the management of HCM 1. The echocardiographic findings of high E/e' ratio and low e' velocities directly indicate impaired diastolic function and elevated left ventricular filling pressures, consistent with the recommendations for the evaluation of left ventricular diastolic function by echocardiography 1. Key points from these guidelines include the use of average E/e′ ratio, LA volume index, pulmonary vein atrial reversal velocity, and peak velocity of TR jet by CW Doppler to evaluate diastolic function in patients with HCM. The steep velocity of propagation of mitral inflow might seem contradictory but can occur in mixed pathologies. These patients often experience symptoms of heart failure despite normal EF because the heart cannot fill adequately during diastole, leading to reduced stroke volume and cardiac output. Management typically focuses on treating the underlying conditions, controlling heart rate to allow adequate filling time, and carefully managing preload and afterload with medications such as beta-blockers, calcium channel blockers, or careful diuresis depending on the predominant pathology, as recommended in the 2024 guidelines for HCM management 1. Some key considerations in the management of these patients include:

• Comprehensive 2D echocardiography for diagnosis and follow-up
• Routine assessment of LV systolic and diastolic function, mitral valve function, and presence and severity of LVOTO
• Use of provocative maneuvers to uncover dynamic LVOT obstruction
• Individualized treatment plans based on the patient's specific condition and symptoms.

From the Research

Presentation Consistency

The presentation of a patient with Hypertrophic Cardiomyopathy (HCM), constrictive pericarditis, and diastolic dysfunction can be complex, with various clinical and echocardiographic findings. Considering the provided evidence, we can assess the consistency of the patient's presentation with the following characteristics:

• Normal Ejection Fraction (EF)
• Low Cardiac Output (CO)
• Mild Left Ventricular Outflow Tract (LVOT) obstruction
• Steep velocity of propagation of mitral inflow
• High E/e' ratio
• Low e' velocities

Echocardiographic Findings

The patient's echocardiographic findings, such as a normal EF, mild LVOT obstruction, steep velocity of propagation of mitral inflow, high E/e' ratio, and low e' velocities, are consistent with HCM and diastolic dysfunction 2, 3. The high E/e' ratio and low e' velocities indicate impaired diastolic function, which is common in HCM patients 2, 3.

Constrictive Pericarditis

The presence of constrictive pericarditis can further complicate the patient's presentation. Constrictive pericarditis can lead to impaired diastolic filling, which can result in low cardiac output 4. The steep velocity of propagation of mitral inflow can be seen in constrictive pericarditis, as it is a marker of enhanced ventricular dependence 4.

Clinical Presentation

The patient's clinical presentation, including low cardiac output, can be consistent with the combination of HCM, constrictive pericarditis, and diastolic dysfunction. The low cardiac output can be due to the impaired diastolic filling and the obstructive effects of the LVOT obstruction 5, 6.

Key Points

• The patient's presentation is consistent with HCM, constrictive pericarditis, and diastolic dysfunction.
• The echocardiographic findings, such as a normal EF, mild LVOT obstruction, steep velocity of propagation of mitral inflow, high E/e' ratio, and low e' velocities, are consistent with HCM and diastolic dysfunction.
• Constrictive pericarditis can further complicate the patient's presentation, leading to impaired diastolic filling and low cardiac output.
• The clinical presentation, including low cardiac output, can be consistent with the combination of HCM, constrictive pericarditis, and diastolic dysfunction, as supported by studies 4, 2, 3, 5, 6.