# ST-segment elevation myocardial infarction in Nail–Patella syndrome with anomalous coronary anatomy and aneurysms: a case report

**Authors:** Kyle Varkoly, Akarsh Parekh, Jason Kaplan, Michael Blair DeYoung

PMC · DOI: 10.1093/ehjcr/ytae188 · European Heart Journal: Case Reports · 2024-04-18

## TL;DR

A rare case of heart attack in a patient with Nail–Patella syndrome shows unusual coronary artery issues and successful treatment.

## Contribution

First reported case of coronary anomalies and successful revascularization in a Nail–Patella syndrome patient.

## Key findings

- Patient with Nail–Patella syndrome had anomalous coronary anatomy and aneurysms leading to heart attack.
- Successful stent placement in overlapping fashion resolved occlusion without complications.
- Highlights potential for systemic vascular anomalies in Nail–Patella syndrome due to collagen alterations.

## Abstract

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.

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.

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.

## Linked entities

- **Diseases:** Nail–Patella syndrome (MONDO:0008061), end-stage renal disease (MONDO:0004375), ST-segment elevation myocardial infarction (MONDO:0041656)

## Full-text entities

- **Diseases:** chest pain (MESH:D002637), end-stage renal disease (MESH:D007676), coronary anatomy (MESH:D003323), genetic disorder (MESH:D030342), renal involvement (MESH:C565423), vascular anomalies (MESH:D020785), anomalous coronary arteries or aneurysms (MESH:D000080038), autosomal-dominant pleiotropic condition (MESH:C566739), pelvic and skeletal abnormalities (MESH:D034161), myocardial infarction (MESH:D009203), NPS (MESH:D009261), coronary artery occlusion (MESH:D054059), ST- (MESH:D000072657), emergency department (MESH:D004630), aneurysmal (MESH:D000783)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

6 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11071451/full.md

## References

15 references — full list in the complete paper: https://tomesphere.com/paper/PMC11071451/full.md

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Source: https://tomesphere.com/paper/PMC11071451