Complex Clinical Cases
The ACC is no longer accepting Complex Clinical Case submissions. Accepted cases will be featured at ACC.22, where presenters will share their science with colleagues from around the world.
Submissions were accepted in the following categories:
- Fellow in Training (FIT) Cases: FIT Cases will be peer-reviewed and selected for presentation in one of two formats — during a special "Stump the Professor" session at ACC.22 or as a traditional or moderated poster. FITs should submit an interesting case based upon the clinical decision-making used to arrive at a diagnosis and/or treatment. The case scenario must illustrate clinical decision-making with teaching points. The first author must be a medical student or anyone in a fellowship or residency program and the presenter must be a Fellow in Training (FIT) to qualify for this category.
- Cardiovascular Team Cases: Submit an interesting clinical case highlighting collaboration and consultation with interdisciplinary colleagues. Highlight the diagnostic features, care coordination/management strategies and experts involved in managing the case and securing a diagnosis. Relate your experience and share with learners at least one clinical pearl that could be immediately replicated in similar practices. CVT cases are peer reviewed. The first author of the CVT Case must be a non-physician CV team member, which could include nurses, advanced practice nurses, pharmacists, physician assistants, CV practice administrators, technologists, registered dietitian nutritionists or exercise specialists/physiologists. The presenter must be a CVT member.
- MD/PhD Cases: Case submissions should be an interesting case based upon the clinical decision-making used to arrive at a diagnosis and/or treatment. The case scenario must illustrate clinical decision-making with teaching points. These cases will be peer-reviewed. The first author and presenter must be a medical doctor or researcher.
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Sample Cardiovascular Team Cases
Case #1Publishing Title: Right Ventricle With the Assist — Applying the Hybrid Concept as a Bridge to Transplant in Infants Without Hypoplastic Left Heart Syndrome
Abstract Body: Background: Options for management of neonates in severe decompensated heart failure are limited, with high morbidity and mortality associated with mechanical circulatory support in this population. The hybrid approach secures systemic circulation via a ductal stent (DS), and protects the pulmonary vasculature via bilateral pulmonary artery bands (PAB). It is typically used as a bridge to transplant or to further palliation in high risk infants with single ventricle congenital heart disease.
Case: A term neonate presented at birth in respiratory failure with severe left ventricular (LV) dysfunction, preserved right ventricular (RV) function, and moderate mitral regurgitation (MR). Troponin I was elevated (5.4 ng/ml) and the electrocardiogram showed ischemia. Coronary angiography revealed left main coronary artery thrombosis. Subarachnoid hemorrhage precluded thrombolytic therapy. On day of life (DOL) 5, he underwent coronary angioplasty. He remained intubated on dual inotropes and prostaglandin E1 with severe LV dysfunction, severe MR, left atrial hypertension with a restrictive atrial septal defect, and retrograde filling of the aortic arch from the patent ductus arteriosus. The patient deteriorated on DOL 21 with pre & post-ductal hypoxemia and lactic acidosis, which resolved after balloon atrial septostomy. On DOL 25, he underwent DS and bilateral PAB. He was extubated on post-operative day 7 and is awaiting transplant on milrinone without respiratory support, and tolerating enteral nutrition.
Decision‐making: In our patient, severe LV dysfunction and MR resulted in left atrial hypertension and respiratory failure. In the presence of severe LV dysfunction, a restrictive atrial level shunt resulted in inadequate systemic output. Balloon atrial septostomy decompressed the left atrium, improving systemic output provided by the RV. DS allowed for stable systemic circulation and bilateral PAB protected the pulmonary vasculature against excessive pulmonary blood flow.
Conclusion: We report a case of a critically ill infant with ischemic LV cardiomyopathy in whom the hybrid concept was successfully used to provide hemodynamic stability while awaiting heart transplant.
Publishing Title: First Report of Subcutaneous Hyaluronidase For Rapid Resolution of Forearm and Hand Hematoma Caused by Cangrelor IV Infiltration and Access Site Bleeding, Following Transradial Approach (TRA) Percutaneous Coronary Intervention (PCI)
Abstract Body: Background: TRA for PCI is associated with less vascular complications and bleeding than femoral approach. Forearm/hand hematomas represent infrequent but potentially serious complications, and occur more often in the setting of intensive antithrombotic therapy.
Case: A 67 yr old NSTEMI patient underwent right TRA PCI with 6 stents deployed and cangrelor infusion via peripheral IV in the right hand. Hemostasis was achieved with a radial compression band but soon after, significant swelling and impaired motor function was noted in the right hand/forearm, due to hematoma plus infiltration of the cangrelor IV infusion. After no improvement with ice/elevation, hyaluronidase, a recombinant endoglycosidase, was injected subcutaneously (SC) into the dorsum of the hand in 0.1-0.2 mL aliquots (total 1 mL). In <12 hours, the swelling completely resolved and the patient discharged without sequelae. (Figure 1)
Decision‐making: After a serious forearm/hand hematoma failed standard therapies, the CCU multidisciplinary team (cardiology, nursing, pharmacy) devised an alternative strategy. While no data addressed this specific scenario, hyaluronidase, classified by the FDA as a diffusing substance which modifies the permeability of connective tissue, has been used to manage other types of extravasations/collections and thus, was trialed.
Conclusion: We describe a novel application of SC hyaluronidase injection which arose from a multidisciplinary approach to a potentially serious vascular complication.
Sample FIT Cases
Publishing Title: Watch For the Watchman's Complication: Atrial Septal Hematoma Mimicking as Left Atrial Thrombus
Abstract Body: Background: Atrial septal hematoma (ASH) is a rare complication after watchman (WM) placement. It could be easily confused with Left atrial (LA) thrombus which is the most common complication of WM implantation.
Case: An 84-year-old female with atrial fibrillation and recurrent gastrointestinal bleeding had a 27mm WM device implanted via transseptal approach. On post-operative day 0 she developed chest pain and pericardial effusion requiring pericardiocentesis. Post-procedural CT chest angiogram showed a 6.3 x 4.9 x3.7 cm filling defect in the LA consistent with thrombus. She was started on heparin drip however, hemoglobin dropped from 11.4 to 8.0 gm/dl overnight without overt source.
Decision‐making: Deciding to anticoagulate or not was the biggest dilemma. A transesophageal echocardiogram (TEE) was repeated and showed a bulky atrial septum and a mobile membrane encasing heterogenous density which was absent on the preoperative TEE. Given the above findings it was concluded that the presumed LA thrombus was actually an iatrogenic septal hematoma from the WM placement. Heparin was discontinued. At 3 months follow up, she had no embolic or thrombotic events and TEE showed complete resolution of the hematoma.
Conclusion: Unlike LA thrombus, atrial septal hematoma is a rare complication post WM placement. A high degree of suspicion and diagnostic modalities such as TEE are required to establish the diagnosis. It is extremely important to distinguish the two as the treatment of one is detrimental to the other.
Publishing Title: Neonatal Myocardial Infarction
Abstract Body: Background: Neonatal myocardial infarction (MI) is rare with high morbidity and mortality. We describe the safe use of transcatheter recombinant tissue plasminogen activator (rTPA) in a patient undergoing cooling for hypoxic-ischemic encephalopathy (HIE).
Case: A full-term male born via emergent cesarean section required 25 minutes of resuscitation and was transferred to our institution for management of HIE. Cooling protocol was initiated. Echocardiogram showed severely depressed left ventricle (LV) function with estimated LV ejection fraction of 8% and with no flow seen in the left coronary artery. Coronary angiography, at 25 hours of life, revealed near occlusive thrombus in the left anterior descending artery (LAD) and complete occlusion of the left circumflex artery (LCx). Three doses of 0.1/mg/kg rTPA were administered into the left coronary artery origin. Final angiogram showed improvement in LAD flow and reestablished flow in LCx with residual thrombus. Low-dose heparin infusion and prostaglandin were continued. Two days later, he underwent catheterization with successful balloon atrial septostomy (BAS). Coronary angiography revealed resolution of LAD thrombus but continued complete occlusion of the LCx. Systemic rTPA was started upon completion of cooling for 4 days. LV function improved to 30-40%. Prostaglandin was stopped at 1 week; he was extubated at 1 month. Serial brain imaging was negative for intracranial hemorrhage. He was discharged at 8 weeks old.
Decision‐making: We describe the safe use of transcatheter rTPA therapy in a neonate with HIE to treat MI. Despite the severely depressed LV function, neonatal ECMO was avoided by performing BAS and maintaining the ductus arteriosus. This strategy allowed the right ventricle to support the systemic circulation until LV recovery had been achieved. The additional benefit of BAS was to decompress the LV and accelerate LV recovery.
Conclusion: Neonatal MI is a rare cause of acute heart failure. Our case highlights the importance of prompt recognition and the safe use of intracoronary rTPA infusion in a neonate with HIE. Reestablishing coronary artery flow is a life-saving procedure and key in recovering devastated heart function.
Sample MD/PhD Cases
Publishing Title: Use of Canakinumab (Illaris) For the Management of Autoimmune Mediated Recurrent Pericarditis
Abstract Body: Background: Recurrent pericarditis (RP) is a common complication after an initial attack of acute pericarditis. Here we describe a case of refractory autoimmune RP.
Case: 31-year-old female first diagnosed with acute pericarditis in 2011. Past medical history of rheumatoid arthritis and ulcerative collitis (UC). The etiology of her pericarditis was noted to be her systemic autoimmune disease. Hence, started on colchicine, prednisone and nonsteroidal anti-inflammatory drug (NSAID). However her symptoms persisted despite triple therapy. Cardiac Magnetic resonance imaging which was significant for pericardial thickening and mild delayed enhancement suggestive of chronic pericarditis. Patient continued to have episodes of recurrent chest pain.
Decision‐making: She commenced on Vedolizumab, then switched to Adalimumab and finally Anakinra was tried but patient only received minimal relief with the aforementioned therapies. Patient continued to have active inflammation secondary to underlying autoimmune process. After a collaborative decision by both the cardiology and rheumatology team, Canakinumab was introduced. After a few months of therapy, repeat imaging demonstrated improved pericardial delayed enhancement and no edema.
Conclusion: NSAIDs, colchine and steroids still remain the mainstay therapy for treatment of RP. However, in a subgroup of patients with autoinflammatory syndromes, Canakinumab potentially could be a promising therapy for autoimmune medicated RP in the future.
Publishing Title: Typical Rheumatic Heart Disease on Echocardiogram. Or is it?
Abstract Body: Background: Mitral stenosis is commonly caused by rheumatic heart disease. Nonetheless, other rare causes of mitral stenosis should be considered in the differential diagnosis.
Case: A 28-year-old man underwent echocardiography as part of perioperative evaluation. Echocardiogram showed thickening and restricted motion of the mitral valve leaflets (hockey-stick deformity). Aortic valve leaflets were thickened with dooming of the distal portion of the leaflets. There was moderate mitral stenosis and regurgitation, and mild aortic stenosis and regurgitation. This pattern is considered characteristic of rheumatic heart disease. After careful history, the patient was found to have Mucopolysaccharidosis type II, Hunter syndrome.
Decision‐making: Hunter syndrome is a rare X-linked recessive lysosomal storage disease that is usually diagnosed in childhood after measuring Iduronate 2-sulfatase (I2S) activity followed by genetic testing. Patients often develop multisystemic disease. Cardiac involvement affects up to two-thirds of patients and it resembles rheumatic heart disease.
Conclusion: Although rheumatic heart disease is the most common cause of mitral stenosis, it is important to consider other rare causes including Hunter syndrome, malignant carcinoid tumor, systemic lupus erythematosus, rheumatoid arthritis, Fabry's disease, Whipple disease, and chronic use of drugs that stimulate the 5HT2b receptor such as methysergide, as this has prognostic and treatment implications.