Description

A man in his 40s with a history of bicuspid aortic valve and atrial flutter was admitted to the cardiac intensive care unit with cardiogenic shock in the setting of rapid atrial flutter. He was treated with diuresis and inotropic support, resulting in improved symptoms and haemodynamic stability. Inotropic support was weaned off, and the patient was transferred to the medical ward for further evaluation and management. At that time, echocardiography showed a left ventricular ejection fraction of 50%, improved from 25% on admission, as well as normal left ventricular size, and a mildly sclerotic native bicuspid aortic valve with mild aortic regurgitation. Physical examination demonstrated vigorous carotid pulsations, known as Corrigan’s pulse (online supplemental video 1), and alternating flushing and blanching of the nail bed in concert with the cardiac cycle, known as Quincke’s pulse (online supplemental video 1). These findings are classically associated with aortic insufficiency. However, echocardiography demonstrated only mild aortic regurgitation, not severe enough to explain the physical findings. Evaluation of the peripheral pulses revealed radial-femoral delay (online supplemental video 1) as well as differences in blood pressure between the upper and lower extremities (left arm, 130/55 mm Hg; left leg, 115/75 mm Hg; right arm, 118/67 mm Hg; right leg, 103/75 mm Hg). Retrospective evaluation of chest radiography showed rib notching, known as Roesler’s sign (figure 1). These findings were consistent with coarctation of the aorta (CoA), and cardiac MRI showed a compatible abnormality within the aortic arch, although it was incompletely evaluated. Subsequent cardiac-gated CT angiography further defined the abnormality to be a type A interrupted aortic arch (IAA) (figure 2) with large chest wall collaterals supplying the descending aorta (figure 3).

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Figure 1

Rib notching is demonstrated on chest radiography in an adult patient with interrupted aortic arch (arrows).

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Figure 2

Sagittal view of cardiac CT angiography shows the location of an interrupted aortic arch (arrow).

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Figure 3

Coronal view of cardiac CT angiography shows hypertrophy of collateral blood vessels in the setting of uncorrected coarctation of the aorta, including the internal mammary arteries that lead to rib nothing (red arrows) and the superior epigastric arteries (yellow arrows).

IAA is considered the most severe form of CoA, in which there is complete anatomical disruption between the ascending and descending aorta. It is uncommon, accounting for only 1.5% of all congenital heart defects. The vast majority of patients present within the first two weeks of life, when the ductus arteriosus closes. Rarely, undiagnosed patients can reach adulthood, and careful clinical evaluation is necessary to make the diagnosis. A variety of therapeutic procedural and surgical techniques are available, including balloon angioplasty, stenting and end-to-end anastomosis, which usually result in excellent outcomes. The treatment of choice depends on patient-specific factors, including age and anatomy.1 This patient underwent cardiac catheterisation that measured a 45 mm Hg gradient across the aortic disruption, with a blood pressure of 127/59 mm Hg in the ascending aorta and a blood pressure of 82/57 mm Hg in the descending aorta. During the procedure, there was an attempt to establish a small channel within the two segments of the aorta in order to perform dilation or stenting of the aortic arch to decompress the collateral vessels and improve blood flow to the lower body, but it was not successful. The patient is currently awaiting evaluation by cardiothoracic surgery for consideration of surgical intervention. He also underwent a successful ablation procedure for atrial flutter. He remains asymptomatic, and repeat echocardiography revealed recovered left ventricular ejection fraction. However, during his most recent clinic visit, he was noted to be hypertensive (left arm, 164/66 mm Hg; left leg, 128/93 mm Hg; right arm, 150/68 mm Hg; right leg, 118/93 mm Hg), a common manifestation of untreated CoA.

Corrigan’s pulse and Quincke’s pulse are commonly seen in patients with chronic, severe aortic regurgitation, and occur when a rapid diastolic collapse in arterial pressure (from backflow of blood into the left ventricle) leads to a large systolic stroke volume (from increased preload).2 3 In this case, cardiac MRI confirmed the aortic regurgitation to be mild, with a regurgitant fraction of 26%, which is not severe enough to cause Corrigan’s pulse or Quincke’s pulse. These physical findings have also been described in the context of high output physiology from other conditions such as cirrhosis, thyrotoxicosis and wet beriberi.4 However, in this case, there was no evidence of increased stroke volume on cardiac MRI. Hyperkinetic peripheral pulses, including Corrigan’s pulse, have been described in CoA, in which there is high blood flow and pressure proximal to the narrowed segment of the aorta, giving rise to vigorous peripheral pulses in the associated distribution.5 To our knowledge, this is the first time Quincke’s pulse has been reported in a patient with CoA. In the absence of an alternative explanation, the observation of Corrigan’s pulse, Quincke’s pulse or other hyperkinetic physical findings should prompt consideration of this condition. Radial-femoral pulse delay occurs when obstruction of the aorta distal to the left subclavian branch results in high blood flow and pressure proximal to the site of coarctation followed by abnormally low blood flow and pressure distal to the coarctation. It is best appreciated by simultaneous palpation of the radial and femoral arteries. Rib notching develops over time due to erosions of the bone caused by increased blood flow and pressure through dilated intercostal arteries that have developed to supply collateral flow to the post-coarctation segment of the aorta.6 Thoracic imaging, such as CT, can be used to confirm the diagnosis and define the lesion for the purposes of surgical planning.