Angina Associated With Left Main Coronary Artery Compression in Pulmonary Hypertension

doi:10.1016/j.healun.2008.12.008

The Journal of Heart and Lung Transplantation

Volume 28, Issue 5, May 2009, Pages 527-530

https://doi.org/10.1016/j.healun.2008.12.008 Get rights and content

Chest pain is a common complaint in patients with pulmonary arterial hypertension (PAH). Left main coronary artery (LMCA) compression by an enlarged pulmonary artery trunk (PAT) has been associated with angina, but appropriate diagnostic and treatment approaches remain poorly defined. We present two cases of angina caused by LMCA compression from an enlarged pulmonary artery, one of which also presented with new, severe left ventricular systolic dysfunction attributed to myocardial ischemia. Diagnosis of LMCA stenosis was made via coronary angiography followed by computed tomography–gated coronary angiography (CT-CA), which confirmed pulmonary artery enlargement as the source of extrinsic compression. Restoring LMCA patency with percutaneous intervention and/or aggressive treatment of pulmonary hypertension led to significant improvement in angina, cardiac function and quality of life. Given the negative impact on cardiac function, prompt diagnosis and treatment of extrinsic LMCA compression should be considered a priority.

Section snippets

Case 1

A 58-year-old man with a history of CREST syndrome (i.e., calcinosis, Raynaud syndrome, esophageal dysmotility, sclerodactyly, telangiectasia) and severe PAH presented complaining of angina. Transthoracic echocardiogram (TTE) indicated a severely dilated and dysfunctional right ventricle (RV), RV systolic pressure of 85 mm Hg, and a markedly enlarged pulmonary artery trunk (PAT) immediately adjacent to the take-off of the LMCA (Figure 1a). Computed tomography–gated coronary angiography (CT-CA)

Discussion

LMCA compression by an enlarged PA is a potentially life-threatening condition that may lead to refractory angina and persistent myocardial dysfunction.⁴ Given that 26% of fatalities attributed to PAH are related to sudden cardiac death,⁷ restoring coronary flow could potentially reduce mortality in PAH.

The incidence of LMCA compression in PAH is not well established. Although early reports estimated its incidence at 44%,⁸ subsequent studies showed an incidence ranging from 4.5% to 20%.⁹ This

References (18)

M. Gomberg-Maitland et al.
United States validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR)
J Heart Lung Transplant
(2008)
J.F. Patrat et al.
Left main coronary artery compression during primary pulmonary hypertension
Chest
(1997)
S. Rich et al.
Stenting to reverse left ventricular ischemia due to left main coronary artery compression in primary pulmonary hypertension
Chest
(2001)
S.M. Mesquita et al.
Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension
Am J Med
(2004)
K. Fujiwara et al.
Left main coronary trunk compression by dilated main pulmonary artery in atrial septal defectReport of three cases
J Thorac Cardiovasc Surg
(1992)
S.M. Kawut et al.
Extrinsic compression of the left main coronary artery by the pulmonary artery in patients with long-standing pulmonary hypertension
Am J Cardiol
(1999)
S.C. Wouters et al.
Preoperative prediction of early mortality and morbidity in coronary bypass surgery
Cardiovasc Surg
(2002)
S. Rich et al.
Primary pulmonary hypertensionA national prospective study
Ann Intern Med
(1987)
W.N. Viar et al.
Chest pain in association with pulmonary hypertension; its similarity to the pain of coronary disease
Circulation
(1952)

There are more references available in the full text version of this article.

Cited by (25)

Lung transplantation for pulmonary hypertension with giant pulmonary artery aneurysm
2020, Journal of Thoracic and Cardiovascular Surgery
Citation Excerpt :
In patient 5, stenting of the left anterior descending coronary artery had been performed before transplantation. This was necessary due to compression of the vessel, a previously described problem in PAH with an enlarged PA.21-24 In the setting of lung transplantation, this is an important issue, because platelet inhibitors add a complicating factor to already highly complex circumstances. Thus, if stenting becomes necessary, it is important to avoid drug-eluting stents and the associated prolonged antiplatelet treatment.
Aneurysm of the main pulmonary artery trunk (PAA) is a rare but severe complicating factor in patients suffering from pulmonary arterial hypertension (PAH). Many centers consider PAA an indication for a heart-lung transplantation. We aimed to summarize our institutional experience with a lung-only strategy in this complex group of patients.
We performed a retrospective single-center analysis of patients with PAH and a severe PAA who underwent lung transplantation between January 1996 and November 2018.
A total of 127 patients with PAH underwent lung transplantation during the study period. Seven patients presented with severe PAA (mean diameter, 70.4 mm). Donor lungs were procured together with the main pulmonary artery (PA). In the recipient, cardiopulmonary bypass with bicaval cannulation was established, and bilateral pneumonectomy together with resection of the entire PA trunk was performed. The right donor lung was implanted, and the attached PA trunk was pulled through behind the superior vena cava and ascending aorta. Anastomosis was performed just above the level of the pulmonary valve. Thereafter, the left lung was implanted by reconnecting the left PA to the main PA trunk. All but 1 patient, who died from sepsis on postoperative day 13, were successfully discharged.
To the best of our knowledge, this is the largest published experience of patients with PAH and severe PAA who underwent lung transplantation. We show that these patients are eligible for double lung transplantation and do not require heart-lung transplantation.
Left Main Coronary Artery Compression in Patients With Pulmonary Arterial Hypertension and Angina
2017, Journal of the American College of Cardiology
Citation Excerpt :
Since the first reported autopsy of a patient with LMCA stenosis in 1957 (26), 94 additional clinical cases have been documented to date. Along with 11 small case series describing 46 patients (9,11,18,27–33), reports of LMCA stenosis have mostly been published as individual case studies. In a series of 26 PAH patients with angina, 7 (26.9%) had significant LMCA stenosis by extrinsic compression of the PA (11), supporting our findings.
Left main coronary artery (LMCA) compression is increasingly recognized as a cause of angina in pulmonary arterial hypertension (PAH).
This study aimed to evaluate the prevalence of LMCA extrinsic compression from a dilated pulmonary artery (PA) in patients with PAH and angina or angina-like symptoms, determine the usefulness of screening with computed tomography coronary angiography (CTCA), and assess the safety and efficacy of percutaneous coronary interventions (PCIs).
All patients with PAH and angina or angina-like symptoms attending the center between May 1, 2008, and December 31, 2013, underwent CTCA. Patients with confirmed LMCA stenosis on selective coronary angiography had PCI.
Of 765 patients with PAH, 121 had angina or angina-like symptoms. Ninety-four patients had abnormal CTCA based on the relationship between the PA and the LMCA and underwent selective coronary angiography. LMCA stenosis ≥50% was detected in 48 of the 94 patients. Forty-five patients underwent PCI with stenting, of whom 41 had sustained angina symptom relief. The 3 other patients had surgical PA reduction plasty. Nine months after PCI, 5 patients had LMCA restenosis and PCI was successfully repeated. The best predictor of LMCA stenosis ≥50% was a PA diameter ≥40 mm. Rates for death or double-lung transplant and the composite rates for death, double-lung transplant, or restenosis at 36 months were 5% and 30%, respectively.
The prevalence of LMCA compression in patients with PAH and angina is high. These results suggest that CTCA is indicated in patients with PAH and angina or angina-like symptoms. PCI was well tolerated, improved symptoms, and resulted in favorable long-term outcomes.
Left Main Coronary Artery Stenting to Relieve Extrinsic Compression by a Giant Pulmonary Artery Aneurysm in a Patient with Idiopathic Pulmonary Artery Hypertension
2016, Heart Lung and Circulation
Pulmonary artery aneurysm (PAA) is a rare but lethal disease. We present a female patient with idiopathic pulmonary artery hypertension (IPAH)-related PAA, who suffered from unstable angina pectoris. Multi-detector computed tomography and coronary angiogram revealed extrinsic compression of the left main coronary artery (LMCA) caused by a giant PAA with severe ostial stenosis. Intravascular ultrasound showed an oval-shaped ostium of the LMCA, indicating extrinsic compression. After successful LMCA stent implantation, chest pain was greatly relieved. This case illustrates that beyond right ventricle ischaemia and coronary atherosclerotic disease, LMCA compression by PAA should be considered in the differential diagnosis of angina in patients with IPAH related PAA. In addition, intravascular ultrasound can be used to confirm the diagnosis and guide the stent implantation safely.
A 55-year-old woman with pulmonary hypertension, worsening dyspnea, and chest pain
2014, Chest
A case of acute coronary syndrome caused by extrinsic compression of the left main coronary artery due to pulmonary hypertension
2010, Journal of Cardiology Cases
Stenosis of the left main coronary artery (LMCA) due to extrinsic compression, producing symptoms of myocardial ischemia, is called left main compression syndrome. We report on a 43-year-old male with acute coronary syndrome who developed left main compression syndrome while waiting for a lung transplantation secondary to interstitial pneumonia, but underwent successful LMCA stenting as emergent treatment. Coronary angiography 3 months after the operation showed good stent patency in the LMCA, and the clinical course was favorable.
Pulmonary hypertension: The contribution of MDCT to the diagnosis of its different types
2010, Radiologia
La hipertensión pulmonar (HP) es una enfermedad caracterizada por la progresiva afectación de los vasos pulmonares, lo que produce aumento de las resistencias vasculares y, como consecuencia, fallo ventricular derecho. La lesión vascular es el factor común a un amplio abanico de patologías y su resultado, la HP, un cuadro clínico grave de mal pronóstico, que agrava el curso normal de las enfermedades a las que se asocia (EPOC, colagenosis, sarcoidosis, cardiopatías congénitas o adquiridas…).
El interés por diagnosticarla lo más precozmente posible se debe a que, actualmente, se dispone de fármacos que mejoran la calidad de vida y han disminuido la mortalidad de estos pacientes y que existen posibilidades quirúrgicas para algunos tipos de hipertensión como la tromboembólica crónica o la asociada a algunas cardiopatías congénitas con cortocircuitos izquierda-derecha.
Ante una sospecha clínica de HP los métodos de imagen son los que confirman el diagnóstico, sugieren una causa, ayudan a seleccionar el tratamiento más adecuado y monitorizan la respuesta. En la actual revisión del tema se presenta la aportación de los diferentes métodos de imagen para el diagnóstico de la enfermedad, haciendo especial hincapié en la TC multidetector (TCMD), que ofrece la posibilidad de estudiar con una sola adquisición todos los órganos torácicos.
Se revisan los signos radiológicos de HP y se establecen los criterios radiológicos actuales para etiquetar el tipo de hipertensión, según la clasificación de Dana Point, basados en el estudio de las alteraciones del parénquima pulmonar, mediastino, espacios pleurales y pericardio y en el estudio de las cámaras cardíacas.
Pulmonary hypertension is characterized by progressive involvement of the pulmonary vessels that leads to increased vascular resistance and consequently to right ventricular failure. Vascular lesions are a common factor in a wide spectrum of diseases, and their result, pulmonary hypertension, is a severe clinical condition with a poor prognosis that worsens the normal course of the diseases to which it is associated (COPD, collagen disease, sarcoidosis, and congenital or acquired heart disease).
It is important for pulmonary hypertension to be diagnosed as early as possible because nowadays drugs can reduce mortality and improve the quality of life; furthermore, some types of pulmonary hypertension (e.g., chronic thromboembolism and those associated with some congenital heart diseases like left-to-right shunt) can be treated surgically.
In cases of suspected pulmonary hypertension, imaging methods can confirm the diagnosis, suggest a cause, help choose the most appropriate treatment, and monitor the response to treatment. This review describes the approach to pulmonary hypertension using different imaging techniques; special emphasis is given to the role of multidetector CT (MDCT), which makes it possible to study all the organs in the thorax in a single acquisition.
We review the radiological signs of pulmonary hypertension and the current (Dana Point) radiological criteria for classifying the type of hypertension based on alterations in the lung parenchyma, mediastinum, pleural spaces, and pericardium, as well as on the study of the chambers of the heart.

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: Supported by an American Lung Association postdoctoral fellowship and T32 research training grants (to V.A.d.J.P.), and by the Vera Moulton Wall Center for Pulmonary Vascular Disease (to F.H., J.A.F. and R.T.Z.).

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Case reportAngina Associated With Left Main Coronary Artery Compression in Pulmonary Hypertension

Section snippets

Case 1

Discussion

J Heart Lung Transplant

Chest

Chest

Am J Med

J Thorac Cardiovasc Surg

Am J Cardiol

Cardiovasc Surg

Primary pulmonary hypertensionA national prospective study

Ann Intern Med

Chest pain in association with pulmonary hypertension; its similarity to the pain of coronary disease

Circulation

Case report
Angina Associated With Left Main Coronary Artery Compression in Pulmonary Hypertension