Alport syndrome (AS) is a genetic disorder due to inheritance of genetic mutations which lead to production of abnormal type IV collagen. AS has been associated with renal, auditory, and ocular diseases due to the presence of abnormal alpha chains of type IV collagen in the glomerulus, cochlea, cornea, lens, and retina. The resulting disorder includes hereditary nephritis, corneal opacities, anterior lenticonus, fleck retinopathy, temporal retinal thinning, and sensorineural deafness. Aortic and aortic valve pathologies have been described as extrarenal manifestations of AS in multiple case reports. One case report described intramural hematoma of the coronary artery. We report the first case of true spontaneous coronary artery dissection (SCAD) with an intimal flap as a very rare manifestation of AS. The patient is a 36-year-old female with history of AS with chronic kidney disease, hypertension, and obesity who presented to the emergency room with acute onset of substernal chest pain radiating to her neck and arms. Troponin was elevated, and ECG showed transient 1 mm ST-segment elevation in the inferior leads. Subsequent coronary angiography revealed localized dissection of the left circumflex artery. Percutaneous coronary angioplasty was performed and her symptoms improved. This case illustrates that SCAD may be a manifestation of AS patients with chest pain.
Case Presentation:
An 86 year old man underwent PCI of distal LAD for severe single vessel coronary artery disease identified after a high risk pharmacologic nuclear stress test (evaluation of exertional fatigue prior to abdominal aortic aneurysm surgical repair). ECHO was consistent with preserved LVEF (60-65%), moderate concentric LVH and mild left atrial enlargement. Less than a week after PCI, he presented to the emergency department for NYHA III dyspnea and fatigue. The patient was not taking any negative chronotropic medications. CT angiography of the chest excluded pulmonary edema, pneumonia and pulmonary embolism; repeat limited ECHO was unchanged. EKG showed first degree AV block (PR 400ms, figure 1). Severely prolonged PR interval with otherwise-unexplained exertional symptoms raised suspicion for pseudo-pacemaker syndrome. In the absence of an alternative cause of declining exertional tolerance, a dual chamber pacemaker with short programmed AV delay (<200ms) was implanted. The patient reported resolution of exertional fatigue and dyspnea on one-month followup.
Discussion:
Pseudo-pacemaker syndrome is a rare, infrequently reported, complication of first degree AV block with severely prolonged PR>300ms. P-wave at the end of the preceding T-wave suggests AV dyssynchrony (arrowhead, figure 1). Left atrial contraction against a closed mitral valve led to loss of atrial contribution to cardiac output, and elevated left atrial pressure. These changes, accentuated by physiologic increase in heart rate on exertion, most likely caused symptoms in this patient. It is interesting that AV dyssynchrony in pacemaker syndrome is caused by the pacemaker (VVI pacing) whereas the AV dyssynchrony in pseudo-pacemaker syndrome from severely prolonged PR interval is treated with a pacemaker. In the appropriate clinical picture, it is an indication for dual-chamber pacemaker implantation for first degree AV block without bradycardia or pauses.
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