Age: 23 years
Gender: Male
Occupation: College student
Working diagnosis: Aortic regurgitation, after closure of outlet ventricular septal defect
HISTORY
The patient was born uneventfully at term, but at 18 months a heart murmur was noted. A cardiac workup established the diagnosis of a doubly committed VSD. The interventricular communication was small because the aortic valve leaflet covered the defect. No aortic regurgitation was noted on follow-up echos. By age 6 years, however, echo demonstrated a slight deformity of the right coronary cusp with very mild aortic valve regurgitation. At that time, a surgical procedure was not indicated on the basis of the small shunt and the mild aortic valve involvement. At 9 years old, the right coronary cusp was found prolapsing toward the LV. Soon thereafter the family moved and the patient was lost to follow-up.
The patient came back to cardiac attention when he was 16 years old, still without symptoms, but now a diastolic murmur was audible. Echo showed mild to moderate aortic valve regurgitation.
Surgical repair was carried out 4 months later, and consisted of closure of the VSD through the pulmonary trunk and plasty of the aortic valve by means of the so-called Trusler method. The operation was uneventful and the postoperative course was uncomplicated.
Residual aortic valve regurgitation was mild during the first several months postoperatively, but progressed later. Catheterization 1 year after surgery demonstrated an enlarged left ventricle. The patient was reluctant to undergo further surgery. On annual follow-up echos, however, left ventricular diastolic dimensions increased gradually over the ensuing years, eventually to an LV diastolic dimension of 67 mm. Therefore, the patient was discussed for further treatment.
Comments: In a doubly committed and juxta-arterial VSD, the outlet septum is missing or very hypoplastic, and the aortic valve and the pulmonary valve are in fibrous continuity without an interrupting muscular bar. There is no off-setting present between the two semilunar valves. This type of defect is more common in Asian populations.
Because of the anatomic orientation, the right coronary cusp of the aortic valve is often affected by the presence of the defect. The leaflet may cover the ostium of the defect partially or completely. If this happens, interventricular shunting is diminished or even absent, and pulmonary blood flow is not excessive. Alternatively, deformity of the right coronary cusp over time tends to produce aortic valve regurgitation. Another outcome in the natural history of such a defect is for the defect to become a sinus of Valsalva aneurysm, which may rupture.
The Trusler method of surgical repair of aortic cusp prolapse/regurgitation consists of plication of the elongated edge of the right coronary cusp and suspension of the commissures.
PHYSICAL EXAMINATION
BP 140/40 mm Hg, HR 58 bpm, oxygen saturation 99% on room air
Height 176 cm, weight 68 kg, BSA 1.82 m 2
Surgical scars: A previous median sternotomy scar was present.
Neck veins: Not distended and had a normal waveform
Lungs/chest: Normal vesicular breath sounds were heard with no rales. There was no chest deformity.
Heart: The rhythm was regular. An early 2/6 high-pitched diastolic murmur was heard at the second left intercostal space. The peripheral pulses were accentuated.
Abdomen: Flat and soft, liver and spleen were not palpable, no bruit was present.
Extremities: No edema or cyanosis was present.
Comments: Unless there are other sources of arterial runoff, the patient’s blood pressure (wide pulse pressure and low diastolic pressure) indicates that aortic regurgitation is clinically significant.
In severe aortic regurgitation, peripheral pulses are bounding as the pulse pressure widens (100 mm Hg, in this case). This is because the systolic arterial pressure is raised with an increased systolic volume, and diastolic pressure is low because of aortic valve regurgitation.
The high-pitched early diastolic murmur is due to aortic valve regurgitation. An Austin Flint murmur (a low-pitched apical diastolic murmur due to anterior mitral leaflet deformation by the aortic regurgitant jet) should be sought.
LABORATORY DATA
| Hemoglobin | 13.9 g/dL (13.0–17.0) |
| Hematocrit/PCV | 39.2% (41–51) |
| MCV | 87.9 fL (85–100) |
| Platelet count | 239 × 10 3 /mL (150–350) |
| Sodium | 140 mmol/L (138–145) |
| Potassium | 3.7 mmol/L (3.4–4.9) |
| Creatinine | 0.8 mg/dL (0.7–1.3) |
| Blood urea nitrogen | 15 mg/dL (8–20) |
| Uric acid | 5.3 mg/dL (3.8–8.0) |
| AST | 16 U/L (0–40) |
| ALT | 13 U/L (0–35) |
| T Bil | 1.1 mg/dL (0.2–1.2) |
| Hb A1c | 4.2% (4.3–5.8) |
| FBS | 82 mg/dL (65–110) |
OTHER RELEVANT LAB RESULTS
| HANP | 46 pg/mL (<40) |
| BNP | 25.5 pg/mL (<20) |
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 58 bpm
QRS axis: +70°
QRS duration: 106 msec
PR interval: 0.18 sec
Normal sinus rhythm with LV hypertrophy
Comments: Note that the precordial leads have been taken at half standard, so the dramatic voltages in the precordial leads are understated. The ST elevation in V1–3 offers some nonspecific support for pathologic LV hypertrophy, since voltage evidence of LV hypertrophy is not very reliable in the young.
U-waves are visible in the precordial leads. These can be present in long QT syndromes or various electrolyte disturbances. In this case, they likely relate to LV hypertrophy.
ECHOCARDIOGRAM
OVERALL FINDINGS
The LV was moderately enlarged (LVEDd 65 mm, LVESd 45 mm in parasternal long-axis view) with reasonable contraction (EF 66%). There was no LV hypertrophy. The LA size was normal. The RV was normal in appearance. There was no residual VSD. There was mild pulmonary regurgitation, trivial tricuspid regurgitation, and no mitral regurgitation. There was retrograde flow in the abdominal aorta.
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