Akarsh Parekh

Background: Nail–Patella syndrome (NPS) is an autosomal-dominant pleiotropic condition characterized by pelvic and skeletal abnormalities and most commonly affecting a tetrad of nails, knees, elbows, and iliac horns, the iliac horns being pathognomonic for the condition. The most well-documented extra-skeletal manifestation is renal involvement with alteration in Type III collagen. No documented cases of NPS with anomalous coronary arteries or aneurysms, acute coronary occlusion, or successfully coronary interventions exist in the medical literature. Case summary: A 62-year-old female with a medical history significant for NPS diagnosed 50 years ago presented to the emergency department with a chief complaint of chest pain. She recently developed end-stage renal disease managed with peritoneal dialysis within the last year. Angiography revealed 100% right coronary artery occlusion with an anomalous take-off from the left circumflex artery. She demonstrated diffuse coronary aneurysms in the right coronary artery, mid-left anterior descending artery, and other epicardial vessels. Two drug-eluting stents were placed in overlapping fashion. Following careful apposition, the aneurysmal segment was successfully stented without complication. The patient was discharged without complication 2 days later. Discussion: Our case shows the first reported case of coronary vascular anomalies and successful coronary revascularization in a patient with NPS in the medical literature. Given the recently reported vascular anomalies and known collagen alterations seen in patients with the genetic disorder, clinicians should suspect further systemic vascular anomalies with their own unique therapeutic challenges when encountering patients with this rare genetic syndrome.

Venous air embolism (VAE) occurs when air is introduced into the venous system and subsequently travels into the right heart and pulmonary circulation. VAE mainly occurs from air that is forced by positive pressure or drawn in by negative pressure. We present a rare case of fatal VAE that occurred during a routine dental root canal procedure. A 69-year-old male was undergoing a root canal procedure at an outpatient dental office under local anesthesia. During the procedure, he went into cardiopulmonary arrest. He was resuscitated, and return of spontaneous circulation was achieved. Thoracic computed tomography was performed and revealed large amounts of air within the right ventricle and portal venous system. VAE should be recognized as a potentially fatal complication resulting from routine dental procedures.

Rao A, et al. J Cardiovasc Imaging. 2021 Dec;30:e9. https://doi.org/10.4250/jcvi.2021.0171

Background: Aortocoronary arteriovenous fistula (ACAVF) due to iatrogenic bypass grafting to a cardiac vein is an exceedingly rare complication resulting from coronary artery bypass grafting (CABG) surgery. If not identified in a timely fashion, ACAVF has known significant clinical consequences related to left to right shunting and possible residual myocardial ischemia. Case presentation: An 82-year-old male with a history of CABG, presented with dyspnea. Over the span of 2 years following CABG, the patient experienced progressive exertional dyspnea and peripheral edema. The patient was found to have a new cardiomyopathy with a severely reduced ejection fraction at 30–35%. The patient underwent diagnostic left heart catheterization, and an ACAVF was discovered between a saphenous vein graft and the coronary sinus. The patient underwent successful percutaneous coiling of the ACAVF with no residual flow. Follow-up echocardiography at 3 months revealed restoration of left ventricular systolic function to 50% and significant improvement in heart failure symptoms. Conclusions: ACAVF is an exceedingly rare iatrogenic complication of CABG that may result in residual ischemia from the non-grafted myocardial territory and other sequelae relating to left to right shunting and a high-output state. Management for this pathology includes but is not limited to the use of percutaneous coiling, implantation of covered stents, graft removal and regrafting, and ligation.

Background: Takotsubo cardiomyopathy (TCM) can clinically present as an acute coronary syndrome; however, the former has regional wall motion abnormalities that extend beyond a single coronary vascular territory without any plaque rupture. Takotsubo cardiomyopathy classically involves apical ballooning of left ventricle (LV). It is uncommon for TCM to present as cardiopulmonary arrest (CPA) along with third-degree atrioventricular (AV) block. Case summary: A 63-year-old female, underwent a ventricular fibrillation (VF) CPA. She was defibrillated three times and return of spontaneous circulation (ROSC) was achieved after 37 min. Her post-ROSC electrocardiogram showed non-specific ST-segment changes and T-wave inversions and soon progressed to third-degree AV block. Patient had a transvenous pacemaker placed to pace her heart. Echocardiogram showed an LV ejection fraction of 15–20% with akinesis of the apex and anteroseptum. An echocardiogram repeated 4 days after the cardiopulmonary arrests showed an ejection fraction of 60–65% with hypokinesis of mid anterior and antero-apical hypokinesis. However, the patient still continued to require a pacemaker and hence eventually received a dual-chamber pacemaker/implantable cardioverter-defibrillator for her AV block and ventricular arrhythmia. Discussion: Most commonly TCM presents with chest pain and symptoms of acute myocardial infarction. We present a very rare presentation of TCM associated with VF and CPA along with third-degree AV block. There have handful of case reports documenting TCM causing CPA in some patients and other case reports showing TCM causing high degree AV block. In our patient, TCM was associated with both VF and CPA along with third-degree AV block.