CardioNerds (Amit Goyal & Dan Ambinder) join Lankenau Medical Center cardiology fellows (Gwen McNeill and Shaung Ooi) for some Philly cheesesteaks! They discuss a fascinating case of Embolic Acute Coronary Syndrome from PFO and Pulmonary Hypertension. Dr. John Clark provides the E-CPR and program director Dr. Jeanine Romanelli provides a message for applicants. Episode notes were developed by Johns Hopkins internal medicine resident Tommy Das with mentorship from University of Maryland cardiology fellow Karan Desai.
The CardioNerds Cardiology Case Reports series shines light on the hidden curriculum of medical storytelling. We learn together while discussing fascinating cases in this fun, engaging, and educational format. Each episode ends with an “Expert CardioNerd Perspectives & Review” (E-CPR) for a nuanced teaching from a content expert. We truly believe that hearing about a patient is the singular theme that unifies everyone at every level, from the student to the professor emeritus.
We are teaming up with the ACC FIT Section to use the #CNCR episodes to showcase CV education across the country in the era of virtual recruitment. As part of the recruitment series, each episode features fellows from a given program discussing and teaching about an interesting case as well as sharing what makes their hearts flutter about their fellowship training. The case discussion is followed by both an E-CPR segment and a message from the program director.
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A woman in her early 40s with history of tobacco and prior methamphetamine use presented with acute onset chest pain. She was found to have an elevated troponin, anterior T wave inversions, and apical akinesis on TTE. Coronary angiography showed a coronary embolism in the mid-distal LAD. Attempts to wire the lesion led to distal embolization of the clot, and IVUS showed no underlying arteriosclerosis.
To investigate the etiology of the coronary embolus, a repeat TTE with agitated saline was done that suggested a PFO with right to left flow, as well as decreased RV function with an estimated RVSP of 70 mmHg. The clinical picture was that of a paradoxical coronary embolus, arising from the right (venous) side traveling to the left (arterial) side via a PFO. PFO closure was discussed but not performed given severe pulmonary hypertension with Right to Left shunt. In this circumstance, the PFO functions as a “pop-off valve” for the overloaded RV; closing it risks precipitating acute RV overload and failure. A RHC showed a PA pressure of 70/24 mmHg with mPAP of 40 mmHg, PCWP 5 mmHg, and PVR of 11 woods units. Given concern for idiopathic PH, a vasodilator challenge was done which did not show reactivity, and she was started on ambrisentan and sildenafil. Ultimately, the etiology of her pulmonary hypertension was felt to be due to PAH from prior methamphetamine use vs. idiopathic PAH. On follow-up, her PA pressures and RV function had greatly approved, allowing for safe and successful PFO closure in an attempt to prevent future emboli. Final diagnosis: Embolic Acute Coronary Syndrome from PFO & Pulmonary Hypertension.
2. PFO on TEE with R to L shunt by Doppler
3. TTE bubble after PFO closure
Episode Schematics & Teaching
The CardioNerds 5! – 5 major takeaways from the #CNCR case
- Coronary emboli are an uncommon cause of myocardial infarction. We can think of the etiology of coronary emboli in three major categories: Direct, Paradoxical, or Iatrogenic.
- Direct emboli originate from the left side of the heart: sources include clot (from atrial appendage, apical thrombus), valvular lesion (vegetation, thrombus, fibroelastoma), or left sided cardiac mass (atrial myxoma, rhabdomyosarcoma).
- Paradoxical emboli originate from the right side or systemic venous circulation, and pass from right to left through an atrial septal defect, patent foramen ovale, or pulmonary arteriovenous malformation.
- Iatrogenic emboli occur following procedures such as valve replacement or PCI. Note that iatrogenic is the most common etiology!
- PFOs are present in up to 25% of adults, but are usually clinically insignificant. However, there is increasing evidence that PFO closure is moderately beneficial compared to antiplatelet therapy alone in patients less than 60 years old with cryptogenic, non-lacunar ischemic stroke. PFO closure may particularly benefit those with a large right-to-left shunt or an associated atrial septal aneurysm.
- Remember closing an ASD in the setting of significant pulmonary hypertension can lead to decompensation, as the interatrial connection may be serving as a “pop-off” valve to decompress the RV and maintain cardiac output in the setting of high PA pressures! Specifically, ASD closure is generally contraindicated if the PA systolic pressure is > 2/3 systolic blood pressure, pulmonary vascular resistance > 2/3 systemic vascular resistance, or if a net right-to-left shunt is present.
- Recall that the hemodynamic definitions of pulmonary hypertension changed in 2019. A mean pulmonary artery pressure of 20 or greater confirms a diagnosis of pulmonary hypertension. A PCWP ≤15 mmHg with pulmonary vascular resistance ≥3 woods units suggests pre-capillary pulmonary hypertension, while a PCWP >15 with pulmonary vascular resistance <3 woods units suggests isolated post-capillary PH.
- During a right heart catheterization, a pulmonary vasodilator challenge can be done to identify vasoreactive patients who may respond to calcium channel blockers. This is typically recommended for idiopathic and hereditary PAH. A positive response is defined as a drop in mean PAP to <40 mmHg, with a decrease of at least > 10 mmHg, with unchanged or increased cardiac output. These patients can be started on amlodipine or nifedipine, but should be followed closely as they may not always remain vasoreactive and clinical deterioration is possible!
- Raphael, C. E., Heit, J. A., Reeder, G. S. et al. (2018). Coronary Embolus: An Underappreciated Cause of Acute Coronary Syndromes. JACC. Cardiovascular interventions, 11(2), 172–180.
- Mojadidi, M. K., Zaman, M. O., Elgendy, I. Y. et al. (2018). Cryptogenic Stroke and Patent Foramen Ovale. Journal of the American College of Cardiology, 71(9), 1035–1043.
- Silvestry, F. E., Cohen, M. S., Armsby, L. B. et al. (2015). Guidelines for the Echocardiographic Assessment of Atrial Septal Defect and Patent Foramen Ovale: From the American Society of Echocardiography and Society for Cardiac Angiography and Interventions. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography, 28(8), 910–958.
- Simonneau, G., Montani, D., Celermajer, D. S. et al. (2019). Haemodynamic definitions and updated clinical classification of pulmonary hypertension. The European respiratory journal, 53(1), 1801913.
- Stout, K. K., Daniels, C. J., Aboulhosn, J. A. et al. (2019). 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation, 139(14), e637–e697.
CardioNerds Case Reports: Recruitment Edition Series Production Team
Bibin Varghese, MD Rick Ferraro, MD Tommy Das, MD Eunice Dugan, MD Evelyn Song, MD Colin Blumenthal, MD Karan Desai, MD Amit Goyal, MD Daniel Ambinder, MD