Age: 45 years
Gender: Male
Occupation: Auto industry worker
Working diagnosis: Recurrent coarctation of the aorta
HISTORY
The patient was well at birth but presented in heart failure at 10 days of age. A diagnosis of severe coarctation of the aorta (CoA) was made by physical examination. He was stabilized with medication and underwent a primary repair at 3 years of age. The method of repair was unknown.
He remained well and was followed infrequently until 9 months ago (44 years old), when he was referred to a cardiologist with high right upper-extremity blood pressure obtained on a routine exam. Echocardiography and MRI suggested a significant recoarctation, and he was begun on antihypertensive therapy with quinapril. One week ago, he presented with an initial episode of atrial fibrillation and was successfully cardioverted. Metoprolol was added, and he was referred for further therapy.
He has no other ongoing medical problems. He is married with children. Until 7 years ago he was a regular smoker.
Comments: Newborn CoA with heart failure is now normally addressed with immediate surgery, eliminating some of the morbidity seen in adults with long-standing hypertension. Ductus arteriosus patency would be a temporary measure, if the distal systemic vascular bed perfusion depends on ductal flow. Although speculative, this patient’s coarctation could not have been critical for him to survive to the age of 3. Probably he also developed collateral flow.
The atrial fibrillation may have been related to elevated LA and LV filling pressure due to hypertension or associated aortic valve disease.
CURRENT SYMPTOMS
The patient is otherwise asymptomatic. He completes 45 hours of work each week without difficulty, can walk up two flights of steps without dyspnea, and enjoys hiking and camping.
NYHA class: I
Comments: Most patients with native or recurrent coarctation are asymptomatic. In isolated coarctation with or without mild aortic valve disease, heart failure is rare.
PHYSICAL EXAMINATION
HR 44 bpm, RR 16, oxygen saturation on room air 98%
Height 178 cm, weight 92 kg, BSA 2.13 m 2
Pulses and blood pressures: The right radial and both femoral pulses were of equal volume with a small pulse delay between the brachial and femorals. The left radial pulse had a diminished volume in comparison with all other pulses. None were bounding. The blood pressures were obtained with appropriate-sized manual cuffs and the patient in the supine position. BP 156/70 (right forearm), 115/60 (left forearm), systolic pressure 118 mm Hg (right thigh).
Surgical scar: Left thoracotomy scar
Neck veins: Normal waveform, not elevated
Lungs/chest: Normal breath sounds
Heart: The precordium was quiet, and there was no thrill. The first heart sound was normal with no early systolic ejection click or third or fourth heart sound. The second heart sound was narrowly split. A grade 3 short, harsh systolic ejection murmur was present at the base and could be heard with less intensity in the neck (right carotid area). A long, early diastolic murmur was audible along the left sternal border. A short, harsh systolic murmur was easily appreciated over the thoracic spine, and a very faint, low-pitched continuous murmur was present in the same area.
Abdomen: No enlargement of the liver or spleen
Extremities: All were warm and well perfused with no temperature changes, mottling, or peripheral edema.
Comments: The low HR may be partially related to metoprolol therapy even though the dose is modest.
A recurrent coarctation is suggested by the systolic gradient of 38 mm Hg recorded from the right arm to the right thigh. The presence of equal pulse volumes with a small delay in transmission of the pulse wave may be compatible with a variety of anatomic distortions of the aortic arch but is commonly seen in the presence of arterial collateral formation. The femoral pulses may also be enhanced by the presence of aortic valve regurgitation.
Radiofemoral delay requires the presence of a collateral source of blood flow to the lower body. This is more specific for aortic coarctation than is simply a lower perfusion pressure in a leg, which could be due to local arterial disease.
The absence of an early systolic ejection click is unusual since a bicuspid aortic valve is present in this patient and should be present in at least 50% of coarctation patients. The harsh murmur at the base may reflect turbulence across the coarctation or aortic valve stenosis. The early diastolic murmur is aortic valve regurgitation that cannot be severe since the pulses are not bounding and the diastolic pressure in the right arm is low normal. However, the coarctation may minimize both of these findings, masking more significant aortic regurgitation. The coarctation can also be appreciated as the harsh murmur in the back, and the continuous murmur suggests the presence of some arterial collaterals.
It is unusual to observe alteration of the extremities in all but the most severe coarctations unless there is coexistent peripheral arterial disease.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 46 bpm
PR interval: 215 msec
QRS axis: −60°
QRS duration: 95 msec
Marked sinus bradycardia with first-degree AV block. Left anterior fascicular block. Inverted T-waves inferiorly and leftward. ST segment elevation in V1–4.
Comments: First-degree AV block may be related to the metoprolol, but in this patient, it predated the atrial fibrillation.
The significance of the left anterior fascicular block is unknown.
Abnormal repolarization may be part of a “left ventricular strain pattern,” although there is no additional evidence here for LV hypertrophy. Even without LV hypertrophy, it suggests some abnormal state of the LV muscle. Of course, the changes may reflect coronary artery disease as well.
CHEST X-RAY
FINDINGS
Cardiothoracic ratio: 52%
While the cardiac silhouette was normal in size, there was a left ventricular configuration to the heart. The aortic arch was normal in size and on the left. The main pulmonary artery segment was small compared with the aortic arch. Pulmonary vascular markings were normal. Irregularity to the posterior fourth and fifth ribs was likely postsurgical (postthoracotomy) given the patient’s history of coarctation repair.
Comments: The prominent LV apex suggests some ventricular overload (hypertrophy), usually right when the apex is elevated. Distortion of the aortic arch, commonly present following coarctation repair, is not seen in this image. There is evidence of dilatation of the ascending aorta.
Rib notching is seldom seen before age 5 in patients with aortic coarctation. In looking for rib notching, one looks for sclerosis of the undersurface of the rib rather than an irregularity of the inferior margin.
ECHOCARDIOGRAM
OVERALL FINDINGS
The LV was normal in size and function. The RV was also normal. There was no mitral or tricuspid regurgitation. The atria were of normal size. The coarctation was not seen.
