Left main coronary artery bifurcation coronary intervention in a patient with achondroplasia with multivessel coronary artery disease

Debasish Das; Anindya Banerjee; Abhinav Kumar; Tutan Das; Shashikant Singh; Jaideep Das Gupta; Manaranjan Dixit

doi:10.4103/injms.injms_144_21

CASE REPORT

Year : 2022 | Volume : 13 | Issue : 3 | Page : 188-191

Left main coronary artery bifurcation coronary intervention in a patient with achondroplasia with multivessel coronary artery disease

Debasish Das, Anindya Banerjee, Abhinav Kumar, Tutan Das, Shashikant Singh, Jaideep Das Gupta, Manaranjan Dixit
Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

Date of Submission	18-Dec-2021
Date of Decision	14-Jan-2022
Date of Acceptance	04-Feb-2022
Date of Web Publication	16-Jul-2022

Correspondence Address:
Dr. Debasish Das
Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar - 751 019, Odisha
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injms.injms_144_21

Abstract

We report a rare case of critical coronary artery disease in a 62-year-old male with achondroplasia. There is a paucity of case reports of association of achondroplasia with coronary artery disease. Left main coronary artery (LMCA) bifurcation lesion carries the highest risk of mortality out of all types of coronary artery lesions and patients with achondroplasia have two-fold more mortality as compared to the general population. That is why achondroplasia patients may be having more sudden cardiac death with the most catastrophic LMCA bifurcation lesion before they undergo revascularization. It may be the plausible explanation behind no literature description of LMCA bifurcation lesion in patients with achondroplasia. Our case is the first literature description of LMCA bifurcation lesion with successful intervention in achondroplasia. The present case will also provide a detailed insight into the association of achondroplasia with coronary artery disease without the presence of conventional cardiac risk factors.

Keywords: Achondroplasia, bifurcation, coronary artery disease, intervention

How to cite this article:
Das D, Banerjee A, Kumar A, Das T, Singh S, Gupta JD, Dixit M. Left main coronary artery bifurcation coronary intervention in a patient with achondroplasia with multivessel coronary artery disease. Indian J Med Spec 2022;13:188-91

How to cite this URL:
Das D, Banerjee A, Kumar A, Das T, Singh S, Gupta JD, Dixit M. Left main coronary artery bifurcation coronary intervention in a patient with achondroplasia with multivessel coronary artery disease. Indian J Med Spec [serial online] 2022 [cited 2023 Jan 30];13:188-91. Available from: http://www.ijms.in/text.asp?2022/13/3/188/351060

Introduction

Scarce is known about the association of achondroplasia with coronary artery disease. Patients with achondroplasia and other causes of dwarfism suffer from increased rates of cardiovascular disease relative to the remainder of the population. The median age of presentation of patients with achondroplasia is 55 years. Most of the patients with achondroplasia presenting with coronary artery disease are moderately obese^[1] with an average body mass index of 32 kg/m². The aforesaid patient had both kyphoscoliosis and lordosis. About one-third of the patients with achondroplasia have multivessel coronary artery disease. Aortic dissection, aortic regurgitation, and pulmonary thromboembolism are described to develop in patients with achondroplasia. Critical coronary artery disease in the form of left main coronary artery (LMCA) bifurcation occlusion has not yet been described in patients with achondroplasia. Our case is a rare description of LMCA bifurcation lesion in a patient with achondroplasia in whom successful coronary artery bifurcation intervention was done. Although dwarfism is associated more with coronary artery disease, achondroplasia per se is not associated with more mortality as compared with the normal population.

Case Report

A 62-year-old male nondiabetic, nonhypertensive, nonsmoker with mild obesity without any family history of coronary artery disease presented to the cardiology outpatient department with rest angina for the last 6 h. He was a known case of achondroplasia since childhood with upper segment: lower segment ratio >1 with kyphoscoliosis and lordosis [Figure 1]. During the presentation, he had a blood pressure of 90/60 mmHg in the right arm supine position with a heart rate of 130 beats/min. His all serum chemistries were within normal limits and he was COVID 19 reverse transcription–polymerase chain reaction negative. Electrocardiogram revealed ST elevation in aVR with global ST depression suggestive of LMCA obstructive lesion. Echocardiography revealed regional wall motion abnormality in the form of hypokinesia of the anterior wall with moderate left ventricular systolic dysfunction (ejection fraction – 38%). The patient was subjected to a right transradial coronary angiogram which revealed a critical left main bifurcation stenotic lesion (Medina 1-1-1) [Figure 2] with a focal lesion in the dominant right coronary artery. With noradrenaline support at 0.05 μgm/kg/min infusion, LMCA was engaged with extra backup guide catheter 6F 3.5. The lesion in the left anterior descending (LAD) was crossed with 0.014” Fielder FC wire and the lesion from LMCA to LAD was initially predilated with a 1.5 × 8 mm semicompliant balloon, followed by 2 mm × 10 mm Ryurei semicompliant balloon and 2.5 mm × 10 mm noncompliant balloon. Post balloon dilatation, the lesion yielded with minor dissection. LMCA to LAD was stented with a 3.5 mm × 26 mm drug-eluting stent [Figure 2] with distal TIMI III flow [Figure 2]. Proximal optimization technique (POT) was done with a 5mm × 8mm balloon for easy recrossing of the guide wire into the side branch. Then, the lesion in LCX was crossed with 0.14 Fielder FC wire and the strut of the main branch stent was dilated with a 1.5 mm × 8 mm semicompliant balloon [Figure 2], followed by 2 mm × 10 mm semicompliant balloon. Then, a T and protrusion stenting was done to LCX with 2.5 mm × 16 mm drug-eluting stent [Figure 3]. Post stenting, final kissing balloon inflation was done with 3.5 mm × 10 mm and 2.5 mm × 16 mm semicompliant balloon simultaneously at 10 atm pressure [Figure 3], followed by POT with a 5 mm × 8 mm balloon [Figure 3]. Post bifurcation stenting, LMCA, LAD, and LCX revealed distal TIMI III flow [Figure 3] with no evidence of local dissection or thrombus. In view of cardiogenic shock, focal lesion in RCA was also stented with 3 mm × 24 mm drug-eluting stent with good distal TIMI III flow. Post PTCA, the patient ECG normalized, EF improved and he was discharged in a stable condition. Our case is a rare illustration of LMCA bifurcation lesion with successful revascularization in achondroplasia.

Figure 1: The patient with achondroplasia

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Figure 2: Stenting from the left main coronary artery to left anterior descending and wiring the LCX

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Figure 3: T and protrusion stenting followed by final kissing balloon inflation and proximal optimization technique with good result

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Discussion

Achondroplasia represents a minority of overall population with an average of 10 years of less life span than nondwarfs.^[2] Dwarfism is associated with an increased risk of cardiovascular death due to increased incidence of atherosclerosis. Between the ages 25 and 35, dwarfism is associated with 10 fold more mortality as compared to the normal population.^[3] Achondroplasia accounts for 90% of cases of dwarfism and are most often sporadic in nature. It is due to mutations in G1138A or G1138C in the fibroblast growth factor 3 gene on chromosome 4. It results in short stature with rhizomelic skeletal dysplasia, macrocephaly, small chest, thoracolumbar kyphosis, and lumbar hyper lordosis.^[4] The median age of achondroplasia patients presenting with coronary artery disease is 55 years. There exists a rule of 50 in achondroplasia: 50% have coronary artery disease, 50% present with ST elevation myocardial infarction, 50% have hypertension, and 50% have hypercholesterolemia. Recurrent coronary artery disease is encountered in almost one-third of cases of achondroplasia. The right coronary artery is involved in almost two-third of the cases, followed by LAD coronary artery. Left circumflex coronary artery is involved in 50% of cases. About 20% of patients with achondroplasia have severe aortic stenosis; the exact cause of this association is not known yet. They are more prone to develop aortic dissection and pulmonary embolism. Two-third of the patients with coronary artery disease undergo coronary intervention and one-third undergo coronary artery bypass surgery as most of the patients with achondroplasia present with a severe form of coronary artery disease. In spite of increased incidence of coronary artery disease, post intervention either in the form of coronary intervention or bypass surgery, they have excellent outcomes. Transradial access poses some technical difficulties due to short limbs, elbow angles, and kyphoscoliosis.^[5],[6] Femoral access may need ultrasonographic guidance in some cases.^[7] Sukhavasi et al.^[1] described in patients with achondroplasia with coronary artery disease, right coronary artery is involved in 75% cases, followed by LAD coronary artery in 71% of cases and left circumflex coronary artery in 57% of cases. They described no cases of left main coronary involvement or LMCA bifurcation lesion. Patients with achondroplasia and coronary artery disease have twofold more mortality as compared to the normal population.^[8] As LMCA or LMCA bifurcation lesions are most catastrophic with the highest mortality out of all the types of coronary lesions and patients with achondroplasia have twofold higher mortality than the normal population, LMCA bifurcation lesion in achondroplasia has not been described in the literature so far. Our case is the first literature description of LMCA bifurcation lesion with successful intervention in a patient with achondroplasia. Few case reports exist about successful transradial intervention in achondroplasia. Shorter catheter course sometimes creates difficulty in engaging the coronary arteries. In the event of hemodynamic instability with pulmonary edema, mechanical intubation becomes difficult due to the presence of kyphoscoliosis and small chest. Although patients with achondroplasia have an increased incidence of coronary artery disease, cardiac intervention in those population can be accomplished considerably safely.

Conclusion

Our case is the first literature description of intervention in LMCA bifurcation lesion in achondroplasia. Although dwarfism is associated with multivessel coronary artery disease and achondroplasia per se, it is extremely rare to encounter such LMCA bifurcation lesion in achondroplasia. Although fluoroscopy has some inherent challenges in achondroplasia due to the presence of kyphoscoliosis and lordosis, successful coronary intervention can be accomplished in those patients without any increased risk of cardiovascular mortality.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

None.

Conflicts of interest

There are no conflicts of interest.

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