- Symptoms and signs of heart failure with preserved left
ventricular ejection fraction (LVEF > 50%).
- Presence of an underlying cause of heart failure with preserved
ejection fraction (eg, comorbidities such as hypertension, coronary
artery disease, diabetes, chronic kidney disease; or underlying
valvular heart disease, restrictive cardiomyopathy, or specific
myocardial diseases such as amyloidosis).
- The diagnosis of diastolic heart failure, which is the most
common cause of heart failure with preserved ejection fraction,
requires definite clinical evidence of heart failure, LVEF > 50%,
and objective evidence of LV diastolic dysfunction by echocardiography
or cardiac catheterization.
Heart failure with preserved ejection fraction (HFpEF) is an increasingly common, debilitating syndrome of the elderly, and it
carries a high rate of morbidity and mortality. HFpEF accounts for
nearly 50% of all hospitalizations for heart failure, and
two large epidemiologic studies have confirmed that patients with
HFpEF have a mortality rate that is nearly identical to heart failure
with low ejection fraction.
HFpEF is the preferred term for patients with a normal ejection fraction who have the syndrome of heart failure, because HFpEF highlights
the fact that heart failure is a syndrome and not a distinct clinical or pathophysiologic entity. Many investigators and experts have used the term “diastolic heart failure” for HFpEF
in the past. However, this term is not ideal for two main reasons. First, there is ample evidence that patients with HFpEF have abnormalities in systolic function (as defined by tissue Doppler imaging), and many patients with heart failure and low ejection fraction have abnormal diastolic function. Second, in the clinical setting, patients with heart failure are currently classified into two categories: low ejection fraction (< 50%) and preserved ejection fraction (> 50%). By calling HFpEF “diastolic heart failure,” clinicians may not consider the entire differential diagnosis of HFpEF (of which pure diastolic dysfunction is only one cause). HFpEF has also previously been called “heart failure with preserved systolic function” or “heart failure with normal systolic function.” As stated above, it is now clear that many patients with HFpEF have abnormalities in systolic function; therefore, HFpEF is a better term.
The most recent American Heart Association/American College of Cardiology (AHA/ACC) guidelines have used the term “heart failure with normal ejection fraction.” This term is also not ideal because there is considerable controversy regarding the exact cutoff for a “normal” ejection fraction.
Therefore, HFpEF is a slightly better term and was used in the most
recent Heart Failure Society of America guidelines on the management
of patients with heart failure. Finally, HFpEF has the advantage
of being an easy mnemonic for patients to remember. HFpEF sounds
like “HUFF-PUFF,” which helps patients understand this
disease, in which dyspnea and fatigue are two of the most common symptoms.
Chinnaiyan KM et al. Curriculum in cardiology:
integrated diagnosis and management of diastolic heart failure. Am Heart J. 2007 Feb;153(2):189–200.
Heart Failure Society of America. Evaluation and management
of patients with heart failure and preserved left ventricular ejection fraction. J Card Fail. 2006 Feb;12(1):e80–5.
Hunt SA et al; American College of Cardiology; American Heart Association Task Force on Practice Guidelines; American College of Chest Physicians;
International Society for Heart and Lung Transplantation; Heart Rhythm Society. ACC/AHA 2005 Guideline Update for the Diagnosis and Management of Chronic Heart Failure in the Adult: a report of
the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Update the 2001 Guidelines for the Evaluation and Management of Heart Failure): developed in collaboration with the American College of Chest Physicians and the International Society for Heart and Lung Transplantation: endorsed by the Heart Rhythm Society. Circulation. 2005 Sep 20;112(12):e154–235.
Since HFpEF is heterogeneous, there is no single mechanism that can explain the pathophysiology of the HFpEF syndrome. In some patients
with HFpEF, such as those who have the signs and symptoms of heart
failure due to severe valvular disease or pericardial disease (ie,
constrictive pericarditis), pathophysiology is relatively straightforward
and well-defined. However, in most patients with HFpEF, pathophysiologic
abnormalities cannot be ascribed to a single well-defined mechanism.
Instead, these patients typically have one or more of the following
underlying pathophysiologic processes: (1) Diastolic dysfunction
due to impaired LV relaxation, increased LV diastolic stiffness,
or both; (2) LV enlargement with increased intravascular volume,
which may be due to extracardiac factors such as renal insufficiency; (3)
abnormal ventricular-arterial coupling with increased ventricular
systolic stiffness and increased arterial stiffness. In addition,
left ventricular hypertrophy and coronary artery disease are especially important
in the pathophysiology of patients with HFpEF.
Diastolic dysfunction occurs when the ventricle loses its normal ability to suction blood from the left atrium. When the ventricle
relaxes abnormally, filling is delayed and left atrial emptying
is incomplete. An abnormally stiff ventricle worsens the problem
by also impeding left atrial emptying. The end result is abnormally
high left atrial and LV diastolic pressures. The LV loses its suction
and instead of “pulling” blood from the left atrium
and pulmonary veins, it now relies heavily on left atrial contraction
so that the LV can fill and distend appropriately, and recoil in
systole. This is one reason why atrial fibrillation is tolerated
so poorly in patients with advanced LV diastolic dysfunction with
resultant elevation of left atrial pressure, pulmonary vascular
congestion, and poor cardiac output.
In patients with HFpEF who have substantial diastolic dysfunction as a cause of their symptoms, the pressure–volume curve
is shifted up and to the left (Figure 19–1).
In these patients, even small increases in central blood volume
or vascular (arterial or venous) tone can result in significant
increases in left atrial volume and pulmonary venous pressures.
Patients with an upward and leftward shift in the LV diastolic pressure–volume
relationship tend to have a high relative wall thickness (high LV
mass/volume ratio), increased fibrosis and scar ...