Age: 31
Gender: Female
Occupation: Nurse
Working diagnosis: Aortic recoarctation
HISTORY
When the patient was 5 years old, the diagnosis of coarctation of the aorta was made after a murmur was heard. One year later she underwent three successive operations for repair. At age 13 a bypass tube graft was implanted between the left subclavian artery and descending thoracic aorta. Through adolescence and early adulthood she felt well but had persistent systemic hypertension despite triple drug therapy.
The patient had had two spontaneous abortions. Knowing the history of her coarctation and extensive repair, her obstetrician referred her to a tertiary care center regarding the potential relationship between her coarctation and miscarriages.
Comments: Aortic coarctation accounts for 5% to 8% of all congenital heart defects, and it is more common in males than females by approximately 2 to 1.
There are several operative techniques ( Fig. 27-1 ) for the surgical management of aortic coarctation. These include subclavian flap aortoplasty, end-to-end anastomosis, extended anastomosis, prosthetic patch aortoplasty, interposition graft, and bypass tube graft. As is common, the surgical details of this patient’s prior repairs were lost, which challenges her care as an adult. From the patient’s own recollection, it seemed that most likely she had at least one interposition graft and one bypass tube graft.
Persistent systolic hypertension despite medical therapy in this young patient should suggest recoarctation or residual coarctation. The incidence of recurrent coarctation ranges between 3% and 41% in a survey of 11 major studies, and may occur with all known surgical techniques. No single technique appears to be superior to the others. The risk of recoarctation is associated with smaller patient size or younger age at operation, and the presence of associated transverse arch hypoplasia. Children operated on in infancy or early childhood are at particular risk.
Many patients with recoarctation are asymptomatic and can be identified with MRI screening.
PHYSICAL EXAMINATION
BP 182/79 mm Hg (right arm), 155/83 mm Hg (left arm), 145/75 mm Hg (right leg), 147/78 mm Hg (left leg); HR 50 bpm; oxygen saturation in right hand 98%
Height 165 cm, weight 48.8 kg, BSA 1.5 m 2
Surgical scars: Midline sternotomy scar and a left lateral thoracotomy scar
Neck veins: JVP was not elevated, and the waveform was normal.
Lungs/chest: The chest was clear to auscultation, with normal breath sounds.
Heart: The heart rhythm was regular. There was a normal apical impulse. Auscultation revealed a normal first heart sound, a normally split second with a very soft ejection systolic murmur in the upper left sternal edge with no continuous murmur. There were no murmurs over the posterior thorax. The right and left radial pulses were equal and forceful whereas both femoral pulses were weak and delayed.
Abdomen: There was no organomegaly.
Extremities: No clubbing of the fingers and toes seen, and there was no edema.
Comments: The major clinical manifestation in adults with coarctation of the aorta is a difference in resting systolic blood pressure between the upper and lower extremities (in most cases, the diastolic blood pressures are similar).
The classic findings are hypertension in the upper extremities, diminished or delayed femoral pulses (relative to the radial), and lower or unobtainable arterial blood pressure in the lower extremities (normally the systolic blood pressure is higher in the leg than the arm). The mechanical obstruction to flow is thought to be largely responsible for the elevation of blood pressure in the upper extremities. In addition, renal ischemia may lead to enhanced renin secretion and “secondary” systolic hypertension.
Auscultatory findings vary and depend on the nature of any associated cardiac lesions and hemodynamic adaptations. Approximately 70% of patients have a bicuspid aortic valve, so an ejection click might be expected at this age, but was not present here.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 63 bpm
QRS axis: +58°
QRS duration: 85 msec
Normal sinus rhythm with a normal axis. Voltage criteria for LV hypertrophy are present (RV4–6 > 25 mm, SV2 > 25 mm, SV1 + RV5 > 35 mm). Nonspecific T-wave inversion in V3. One ventricular premature beat is seen.
Comments: The most common finding in coarctation is evidence of LV hypertrophy.
CHEST X-RAY
FINDINGS
Cardiothoracic ratio: 49%
Cardiac size is at the upper limit of normal, with mild dilatation of the ascending aorta. There is no obvious rib notching. The lung parenchyma is normal.
Comments: Rib notching can be present from severe coarctation with extensive collateralization. It is not seen in the anterior ribs because the anterior intercostal arteries are not located in costal grooves.
EXERCISE TESTING
| Exercise protocol: | Modified Bruce |
|---|---|
| Duration (min:sec): | 11:00 |
| Reason for stopping: | Dyspnea |
| ECG changes: | Frequent bigeminy, no ST change |
| Rest | Reak | |
|---|---|---|
| Heart rate (bpm): | 50 | 137 |
| Percent of age-predicted max HR: | 72 | |
| O 2 saturation (%): | 98 | 99 |
| Left arm blood pressure (mm Hg): | 155/85 | 190/90 |
| Right arm blood pressure (mm Hg): | 180/80 | 200/90 |
| Peak V o 2 (mL/kg/min): | 17.3 | |
| Percent predicted (%): | 50 | |
| Ve/V co 2 : | 36 | |
| Metabolic equivalents: | 5.7 | |
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