Pediatric Aortic Valve Insufficiency Clinical Presentation

Presenting symptoms in aortic valve insufficiency include the following:

• Chronic severe aortic valve insufficiency

  • Gradual enlargement of the left ventricle (LV) and gradual increase of stroke volume in most cases

  • No symptoms for many years in most patients with chronic aortic valve insufficiency

  • No symptoms for decades in most patients with chronic moderate aortic valve insufficiency

  • Exertional dyspnea (common)

  • Orthopnea (common)

  • Paroxysmal nocturnal dyspnea (common)

  • Angina pectoris (less common)

  • Abdominal discomfort (less common)

  • Syncope (rare)

  • Nocturnal angina with diaphoresis (rare)

• Causes of symptoms in chronic severe aortic valve insufficiency

  • The onset of myocardial ischemia and diastolic dysfunction

  • Splanchnic ischemia, which may cause abdominal discomfort

• Symptoms associated with acute severe aortic valve insufficiency

  • Chest discomfort

  • Dyspnea

  • Hypotension

  • Uncomfortable awareness of heartbeat/palpitations

  • Chest pain

  • Sudden cardiovascular collapse

• Cause of symptoms in acute severe aortic valve insufficiency

  • Sudden drop in coronary driving pressure

  • Increased filling pressure of the LV

  • Increased left atrial pressure

  • Low cardiac output

Physical examination findings can be associated with the signs and symptoms of various degrees of severity of insufficiency and with the status of LV compensation. These may include physical findings of widening of pulse pressure or volume overload. In chronic aortic valve insufficiency, systolic pressures are abnormally elevated and diastolic pressures are abnormally low, which may indicate the extent of aortic valve insufficiency.

Coexisting cardiac pathology, such as a ventricular septal defect or aortic stenosis, may also be revealed during physical examination.

• Physical findings in chronic severe aortic valve insufficiency

  • Water-hammer pulse - Bounding radial pulse with elevation of the patient's arm

  • Corrigan pulse - A quick filling and collapse of carotid pulse

  • de Musset sign - Head bobbing with each systole

  • Bisferiens pulse - Characterized by 2 systolic peaks of equal or unequal magnitudes separated by a midsystolic trough; usually detected well in the carotid artery, but noted better in the brachial and femoral pulses in severe chronic aortic valve insufficiency

  • Traube sign - Bounding pistol shot–like femoral artery pulse during systole and diastole

  • Müller sign - Pulsations of the uvula during systole

  • Duroziez sign - Murmur heard over femoral artery when compressed; heard during systole when the femoral artery is compressed proximally and heard during diastole when the femoral artery is compressed distally

  • Quincke sign - Pulsations of the nail beds with systole, when the nail is distally compressed

  • Hill sign - A greater than 40 mm Hg elevation of the popliteal systolic pressure over that of the brachial systolic pressure

• Physical findings in chronic aortic valve insufficiency associated with increased stroke volume

  • Displacement of the point of maximal impulse inferiorly and laterally, consistent with the volume overload and increased LV chamber size

  • Hyperdynamic apical impulse

  • Diastolic thrill, consistent with rapid forceful ventricular emptying

  • Systolic thrill over suprasternal notch or carotid arteries, consistent with increased stroke volume

• Auscultatory findings in chronic severe aortic valve insufficiency

  • Soft S₁

  • S₂ abnormalities, which may include absent S₂, single S₂, or paradoxical splitting of S₂

    • Soft or absent A₂ portion of S₂ can be caused by incomplete or abnormal closure of the aortic valve.

    • Absent P₂ portion of S₂ may be caused by the murmur of AI during the early diastole.

    • Paradoxical splitting of S₂ can be caused by delayed closure of the aortic valve because of increased preload volume.

  • Systolic murmur - Caused by forceful ejection of overloaded volume from the LV, which results in aortic distension

  • S₃ - Can be heard when filling of the LV continues through an already expanded and stretched LV; may suggest an extremely high-end systolic volume of the LV and may be an early sign of impeding LV failure and severe AR

  • Diastolic murmur

• Quality of the murmur of chronic aortic valve insufficiency

  • High frequency

  • Begins immediately after A₂

  • Best heard with patient sitting up or leaning forward (increases the setting of preload and volume return to the heart, accentuates the murmur of aortic valve insufficiency)

  • Best heard with deep expiration

  • Holodiastolic decrescendo (signifies severe insufficiency)

• Auscultatory differentiation between chronic severe and chronic mild aortic valve insufficiency

  • Intensity and duration of the murmur correlate with the amount of volume overload and stroke volume ejected from the LV to the aorta.

  • A murmur that is confined to the early part of diastole correlates with mild AR. This murmur is also high pitched.

  • A murmur that extends through diastole correlates with severe aortic valve insufficiency.

  • A musical-type murmur may be related to a perforated aortic valve cusp.

• Determining where the aortic valve insufficiency murmur is best heard

  • A murmur that is best heard at the left sternal border at the third and fourth intercostal spaces suggests a primary valvular disease.

  • A murmur that is best heard along the right sternal border suggests proximal aortic root dilatation.

  • An Austin Flint murmur is a mid-diastolic to late-diastolic rumble, which is best heard at the apex.

• The Austin Flint murmur

  • This mid-diastolic to late-diastolic murmur is present when a surge of flow from the left atrium to the LV is counteracted by the regurgitant flow from the aorta to the LV as it courses back into the mitral valve. The regurgitant flow causes constriction of the mitral opening and accentuates the rumble of the forward flow from the left atrium through the mitral valve.

  • The Austin Flint murmur is similar to the murmur of mitral stenosis. The difference is that S₁ is not as loud in aortic valve insufficiency as it is in mitral stenosis.

  • Onset, duration, and termination of the Austin Flint murmur are related to the amount of LV volume overload and the end-diastolic pressure in the LV chamber. In the most severe form of increased end-diastolic pressure, the murmur is heard in early to mid diastole.

  • The Austin Flint murmur may be graded 1-4, depending on the ejection fraction and stroke volume. The murmur can be transmitted upward to the carotid vessels.

• Certain maneuvers that can alter the intensity of the murmur in chronic aortic valve insufficiency

  • Sitting up or leaning forward causes increased volume overload and increased LV end-diastolic pressure; therefore, it increases the intensity of the murmur.

  • Straining, Valsalva maneuver, and hypotension decrease the intensity of the murmur.

• Physical findings in acute severe aortic valve insufficiency

  • Severe cyanosis

  • Tachycardia

  • Severe dyspnea indicating pulmonary congestion and LV failure

  • Edema

  • Limited peripheral manifestations

  • No significant widening of pulse pressure

  • May relate to acute mitral valve regurgitation and premature closure of mitral valve

  • Pulmonary hypertension with S₃, S₄, and loud P₂

  • Low-pitched short early diastolic murmur: In extremely severe aortic valve insufficiency, when LV decompensation occurs, a significant part of the murmur is diminished as an equalization of pressure occurs between the aorta and the LV in the latter part of diastole.

  • Shortened (or absent) Austin Flint murmur, with no presystolic component because of premature closure of the mitral valve during diastole

See the list below:

• Causes of aortic valve insufficiency include congenital malformations of the aortic valves and diseases of the aortic valves and root.

• See Pathophysiology for further discussion of causes.