Age: 27 years
Gender: Female
Occupation: Business manager
Working diagnosis: Aortic coarctation
HISTORY
The patient was diagnosed with aortic coarctation within the first week of life and was operated on at the age of 2 months. A left subclavian flap angioplasty was performed.
Thereafter, the patient remained asymptomatic and developed normally throughout childhood. She never experienced any chest pain and did not develop systemic hypertension. However, she did not receive regular follow-up.
As an adult the patient expressed an interest in starting a family, and sought advice at our adult congenital heart disease clinic about the safety of pregnancy. A general workup followed, and the patient was told that no interventions were required.
The patient later presented to a high-risk obstetrics service 11 weeks pregnant.
Comments: Generally, the earlier a patient undergoes repair for coarctation, the lower the chance of future complications from hypertension (see also Case 25 ). In contrast, the risk of recoarctation is higher in patients requiring angioplasty repair in infancy compared to those who undergo surgical repair of coarctation later on in childhood or during adult life.
The left subclavian flap angioplasty was first introduced in 1966 by Waldhausen and Nahrwold and involves dividing of the left subclavian artery and ligating it distally. The proximal part of the artery is then split longitudinally, turned down, and used as a flap to enlarge the stenosed portion of the descending aorta. It is used primarily in patients less than 1 year of age.
Prepregnancy counseling should be offered to all women with congenital heart disease to prevent avoidable risks and crisis management. Estimation of the individual pregnancy risk for these women should be based on a recent evaluation of their cardiac status and anatomy.
A patient with previous coarctation repair should be investigated before pregnancy to exclude residual coarctation, aortic aneurysm formation, and systemic hypertension.
CURRENT SYMPTOMS
The patient presented without any symptoms and was feeling entirely well. She denied nausea, vomiting, palpitations, or shortness of breath.
NYHA class: I
Comments: Significant cardiovascular changes occur during pregnancy. There is a steady increase in blood volume and cardiac output until the end of the second trimester, at which time cardiac output reaches a plateau at 30% to 50% above prepregnancy levels. But at the 11th week of gestation, such changes are not yet substantial.
PHYSICAL EXAMINATION
BP 135/70 mm Hg (right arm), BP 110/65 mm Hg (right leg); HR 74 bpm, oxygen saturation 99%
Height 171 cm, weight 56.7 kg, BSA 1.64 m 2
Surgical scars: Left lateral thoracotomy
Neck veins: JVP was not elevated.
Lungs/chest: Chest was clear.
Heart: There was a normal first and a physiologically split second heart sound. There was a soft 2/6 systolic ejection murmur, but there was no diastolic murmur or murmurs over the back.
Abdomen: Normal
Extremities: There was no peripheral edema, and there were normal peripheral pulses palpable at the right arm and both legs without radiofemoral pulse delay. The left radial pulse was not palpable. The left arm was slightly smaller than the right arm.
Comments: An abnormal difference in upper and lower limb arterial pulses and blood pressures is the clinical hallmark of coarctation. Manual cuff blood pressure is normally about 10 to 20 mm Hg higher in the leg than arm. Reversal of this pressure pattern may be suggestive of aortic arch obstruction, a sign that is commonly used for the clinical evaluation of coarctation or recoarctation. In significant cases, pulses distal to the obstruction are diminished and/or delayed. In this patient the pressure gradient between the right arm and the right leg and the soft murmur without an obvious radiofemoral pulse difference or delay were in keeping with a diagnosis of mild residual coarctation.
Diastolic murmurs in the back would be evidence of more severe coarctation, since they indicate the development of collateral circulation to bypass significant aortic obstruction.
The left arm arterial pulse was not palpable. After surgery for aortic coarctation with a subclavian flap, blood flow to the arm is often compromised, as the subclavian artery has been sacrificed (via Waldhausen subclavian flap angioplasty). The right arm is usually the best side to measure the blood pressure proximal to the site of surgery (with the exception of patients with an aberrant right subclavian originating distal to the aortic arch and coarctation).
The loss of the subclavian artery as part of a subclavian flap angioplasty of aortic coarctation in infancy does not usually impact the growth of the left arm, as collateral arterial flow develops, and certainly symptoms of brachial ischemia are exceedingly rare.
CHEST X-RAY
FINDINGS
Cardiothoracic ratio: 44%
The CXR was done 2 years before pregnancy.
There was situs solitus and levocardia (apex pointing to the left). There was a left aortic arch and no cardiomegaly. The aortic knuckle was abnormally prominent. Additionally, there were deformities in the left-sided ribs (ribs 2 to 4). The pulmonary vascular markings were normal.
Comments: This CXR showed some typical findings for a patient late after repair of coarctation. The left-sided deformities of the ribs are a typical result of left lateral thoracotomy in infancy. The prominent aortic knuckle is suspicious for aneurysm formation at the site of previous surgery. There is no obvious dilatation of the ascending aorta, making coexistence of a bicuspid aortic valve less likely.
BLOOD PRESSURE MONITORING
FINDINGS
The average blood pressure recorded in the right arm was 122/74 mm Hg. During the day, the average blood pressure was 127/79 mm Hg and at night 105/59 mm Hg.
Comments: This documented normal systemic blood pressure profile is reassuring and excludes systemic hypertension in this patient.
Systemic hypertension is common in patients with aortic coarctation even after successful repair of this anomaly and without residual mechanical obstruction.
Systemic hypertension can develop or worsen during pregnancy and increases the risk for aortic dissection or rupture of preexisting aneurysms. Furthermore, patients with coarctation of the aorta are at increased risk of preeclampsia. Meticulous blood pressure control is therefore crucial in these patients.
EXERCISE TESTING
| Exercise protocol: | Modified Bruce |
|---|---|
| Duration (min : sec): | 12 : 12 |
| Reason for stopping: | Dyspnea and dizziness |
| ECG changes: | None |
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