A pulse is a rhythmic wave produced by ventricular contraction during systole. A double pulse noticed during systole in the peripheral pulse is called pulsus bisferiens. This is derived from the Latin word, which means strike twice (bis=twice, ferio=strike). It is also called a biphasic wave. Way back in 1952, it was described by Galen in De Pulsibus.
Pulsus bisferiens is associated with the severe aortic disease with aortic regurgitation and HOCM. Providers often confuse this with the dicrotic pulse. The main distinguishing feature of pulsus bisferiens is that two peaks are seen in systole whereas, the dicrotic pulse is characterized by one peak in systole and other in diastole. Dicrotic pulse is seen in the low cardiac output state, sepsis, and cardiac tamponade.
Pulsus bisferiens a single central pulse wave with two peaks separated by a distinct mid-systolic dip. An early component percussion wave results from rapid left ventricular ejection. The late component tidal wave represents a reflected wave from the periphery due to an artery's recoil effect.
Medical literature has made many revelations about the pulse way back in the first and second centuries AD.
The most common causes of pulsus bisferiens are mixed aortic valve disease (infective endocarditis, rheumatic heart disease, Marfan syndrome, bicuspid aortic valve) and hypertrophic cardiomyopathy with obstruction (HOCM).
Other causes are:
Pulsus bisferiens is commonly seen in mixed aortic disease with severe aortic regurgitation and hypertrophic cardiomyopathy.
The mechanism of pulsus bisferiens is not completely understood. A normal central pulse has two waves; a percussion wave (larger) and a tidal wave (smaller). It is not easy to distinguish them clinically, as it happens in quick succession. But in some of the pathological conditions, these are exaggerated by a wider mid-systolic gap leading to two distinct waves.
It is postulated that a large, rapidly ejected left ventricular stroke volume is associated with left ventricular changes in the pressure over time, resulting in percussion wave. The second wave (tidal wave) is caused by the backpressure exerted by the arterial musculature's recoil. Katz et al. (1927) also described the theory of a venturi effect caused by the high flow of blood in the ascending aorta. This theory has not received much of the attention of the researchers.
Age gives crucial clues to the diagnosis of the disease. The pediatric age group with a palpable pulse with two strokes should indicate PDA with a left to right shunt. The younger patient usually points towards a diagnosis of hypertrophic cardiomyopathy with an obstruction or bicuspid valve disease, whereas mixed aortic valve disease is more common in the elderly population. Pulsus bisferiens can be more pronounced by the Valsalva maneuver.
Common clinical presentations are syncope, dyspnea, chest pain, and palpitations. Long-standing problems can remain asymptomatic and later can present with signs of acute pulmonary edema and heart failure. HOCM can present with sudden cardiac death and life-threatening arrhythmias, especially in younger athletes.
On palpation of a peripheral pulse (radial artery), two upstrokes distinguished by a mid-systolic gap in the systole are characteristic of pulsus bisferiens.
Aortic regurgitation can also present with other clinical signs such as blanching and flushing of the forehead and face (Lighthouse sign), bobbing of the head synchronous with the arterial pulse (De Musset sign), repeated flushing and blanching of the capillaries in the nail beds (Quincke sign), alternating systolic miosis and diastolic mydriasis in both pupils (Landolfi sign), pulsations of the retinal arteries on the fundoscopic examination (Becker Sign), pulsations of the uvula (Muller sign), systolic pulsations of the liver (Rosenbach sign) and spleen (Gerhard sign), a pistol shot sound both during systole and during diastole on auscultation (Traube sign), diastolic-systolic murmur over the femoral arteries (Duroziez murmur).
Serum Biomarkers: High sensitivity troponin T (Tn-T) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels are elevated in bicuspid aortic valve disease.
Chest X-Ray: Chest X-ray can reveal LVH, left atrial enlargement, and heart failure (pulmonary edema).
ECG: Conduction abnormalities, AF, left ventricular hypertrophy, and left atrial hypertrophy are seen in both aortic valvular disease and HOCM.
2D-Echo: Echocardiography is the main diagnostic tool for diagnosing mixed aortic valve disease and HOCM. Assessment of valve morphology, determination of the aortic regurgitation jet, aortic regurgitation quantification, measurement of the aortic root and ascending aorta can be done with 2D-echocardiography. Help in left ventricular assessment and measurement of its dimensions in valvular disease and hypertrophic cardiomyopathy. It also can be used to assess the morphology of the aorta and determine the feasibility of aortic surgery or valve repair.
MRI: Cardiac magnetic resonance imaging is increasingly used to assess regurgitant lesion severity and ventricular function. Advances in noninvasive imaging help in objective measurements of valve disease severity, hemodynamic fluctuation, and structural complications. MRI is also used increasingly in the quantification of LV remodeling accurately. Cardiac MRI is the gold standard test in assessing patients with HOCM.
CT: Cardiac and vascular CT has become an indispensable tool for the appropriate planning of transcatheter interventions. Useful in acquiring intracardiac and vascular access route dimensions with accuracy.
Nuclear Imaging and Fluoroscopy: The extent of local infectious, metabolic activity, the extent of calcium deposits can be evaluated with nuclear imaging and fluoroscopy.
Management of pulsus bisferiens is based on its etiology. Early diagnosis and referral to the cardiologists hold the key to success in the management, reducing mortality and morbidity. Mixed aortic valve disease and hypertrophic cardiomyopathy with obstruction are the most common causes of pulsus bisferiens.
An important differential diagnosis for a bisferiens pulse is a dicrotic pulse. It is always difficult to distinguish between these two clinically. In the dicrotic pulse, two peaks are palpable; one in systole and the other in diastole. Whereas in pulsus bisferiens, both peaks are seen in systole. Dicrotic pulse is seen in cardiac failure, sepsis, and occasionally seen in normal individuals after an exercise.
Mixed Aortic Valve Disease: Prognosis is generally good in patients with an isolated severe aortic regurgitation (AR) who are asymptomatic and have a preserved LV function. Patients with moderate to severe AR have a poor outcome. Surgical intervention, aortic valve replacement (AVR) can improve the survival rate in symptomatic patients with LV dysfunction.
Hypertrophic Obstructive Cardiomyopathy (HOCM): The mortality rate is very low for patients with HOCM. Generally, the long-term outcome is good. A small percentage of the population has a risk of sudden cardiac death.
Patent Ductus Arteriosus (PDA): PDA severity scores have been developed, which can be used to predict serious outcomes. Ductus closes spontaneously in most healthy newborns; only 3% of the infants weighing more than 1,000 gms may require a PDA intervention.
Complications in patients who have pulsus bisferiens include:
With the recent advances of transcatheter technologies, valve replacement techniques, and surgical repair, the community must establish institutional-based standards to deliver high-quality cardiac care. Early diagnosis at the primary care level with the involvement With the recent advances of transcatheter technologies, valve replacement techniques, and surgical repair, the community must establish institutional-based standards to deliver high-quality cardiac care.
Early diagnosis at the primary care level with providers, cardiologists, interventional cardiologists, and surgeons with timely referral to the higher center can improve outcomes. With optimal care and follow-up, serious side effects of severe aortic valve disease and HOCM such as heart failure, bacterial endocarditis, ventricular arrhythmias that lead to increased patient morbidity and mortality can be prevented. Multidisciplinary team involvement in the assessment and management of cardiac disease will lead to better outcomes.
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