Age: 26 years
Gender: Female
Lifestyle information: Active athlete and traveler, engaged to be married
Working diagnosis: Aortic insufficiency and neoaortic root dilatation
HISTORY
The patient was born with a tricuspid aortic valve, but as a child she developed aortic valve endocarditis resulting in perforation of the noncoronary cusp. Aortic insufficiency worsened, and at age 9 she required surgery to repair the insufficiency. The procedure was unsuccessful, however, and again at age 10 she underwent repeat surgical repair aortic valvuloplasty, with better results. She was started on lisinopril.
At age 18, aortic insufficiency had progressed and she required further surgery. She underwent a Ross procedure, which was uncomplicated. In early follow-up both her neoaortic root and pulmonary homograft were functioning well.
Eight years after the Ross procedure, she reported a significant decline in her exercise tolerance, growing steadily worse over the past year.
Comments: Endocarditis in the young is uncommon but does occur, with risk factors similar to those in adults. It is more common in patients with a bicuspid aortic valve, or any source of turbulent flow around the valve. Sometimes a tricuspid aortic valve can be “functionally bicuspid” if there is considerable fusion along one of the commissures.
Often valvuloplasty is performed in children or adolescents, even if results are less than optimal, to delay valve replacement until growth is complete.
The Ross procedure reduces the need for future aortic valve surgery (although pulmonary valve redo surgery may be required, see Case 23 ), and avoids the need for long-term warfarin, especially important in a woman of childbearing age.
PHYSICAL EXAMINATION
BP 100/42 mm Hg, HR 100 bpm, oxygen saturation 100%
Height 178 cm, weight 67 kg, BSA 1.82 m 2
Surgical scars: Median sternotomy
Neck veins: The venous waveform was normal.
Lungs/chest: Clear to auscultation
Heart: The precordium was active. There was a 2/6 blowing diastolic murmur heard along the left sternal border. Peripheral pulses were bounding. There was a diastolic flow murmur at the femoral artery.
Abdomen: Soft with no organomegaly or masses
Extremities: Warm and well perfused
Comments: The physical exam suggests worsening aortic insufficiency, from the wide pulse pressure, the early diastolic murmur, the bounding peripheral pulses, and possible flow reversal in the femoral artery.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 110 bpm
QRS axis: Between +30° and +60°
PR interval: 120 msec
QRS duration: 100 msec
Sinus tachycardia. Presence of LV hypertrophy and diffuse ST-T abnormalities.
Comments: This ECG (see Fig. 19-1 ) shows some of the typical characteristics of LV hypertrophy (voltage criteria) as can be found in patients with significant chronic aortic regurgitation. Left precordial Q-waves can reflect chronic volume overload. However, the diffuse ST-T abnormalities are nonspecific and could also be related to myocardial injury from previous cardiac surgery.
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