Abstract
Isolated spontaneous coronary intramural hematoma is a unique subset of spontaneous coronary artery dissection that is characterized by a hemorrhage limited to the medial–adventitial layers, causing subsequent hematoma formation without visible intimal flaps. It is an infrequent and serious coronary vessel wall pathology, with poorly understood underlying pathogenic mechanisms. Affected individuals may present with a broad spectrum of symptoms ranging from acute coronary syndromes (ACS) to cardiogenic shock or even sudden cardiac death. The disease entity causes challenges in terms of both diagnostics and treatment strategy. Coronary intramural hematomas can also occur iatrogenically, as a complication to percutaneous coronary intervention (PCI). Coronary angiography (CAG) has limited diagnostic value in the absence of intimal dissections, and lesions are often angiographically ambiguous. Intravascular ultrasound (IVUS) is an important diagnostic tool in establishing the correct diagnosis, as it provides a complete vessel wall assessment, and enables morphometric information regarding the magnitude and severity of the underlying hematoma. Due to the rarity of this clinical scenario, no randomized, controlled trials exist to guide treatment, and no consensus regarding management is available. Currently, treatment strategies are based on a case-by-case clinical assessment, and experiences described in previous, limited retrospective studies and case reports.
Highlights
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This manuscript addresses a case series of a large spontaneous coronary intramural hematoma occurring in the aftermath after an episode of pronounced gastroenteritis, and two cases of large iatrogenic coronary intramural hematomas occurring as complications after percutaneous coronary intervention with stent implantation.
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The essential diagnostic and treatment-guiding role of intravascular ultrasound is highlighted in the case descriptions.
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Due to the rarity of cases and sporadic reporting of therapy experiences in the literature, no consensus concerning optimal treatment exists.
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In the discussion section of this manuscript a focused literature review summarizing published reports and required therapy is provided.
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Our findings/experiences indicate, that the coronary vessel wall might restitute by itself following isolated spontaneous coronary intramural hematoma, and percutaneous revascularization treatment should be restricted to patients with refractory ischemia.
1
Introduction
Spontaneous coronary intramural hematoma is a unique and rare subset of spontaneous coronary artery dissection (SCAD), where a hemorrhage within the vessel wall is thought to be the underlying cause, leading to separation of the mural layers. The hematoma formation is limited to the medial–adventitial layers, and no flaps are visible when assessed with tomographic techniques . Previously, rupture of the vasa vasorum without communication with the lumen has been proposed as the underlying pathophysiological mechanism . This serious condition usually presents as acute coronary syndrome (ACS), but symptoms may range from chest pain to cardiogenic shock or sudden death , depending on hematoma location, number of affected vessels, lumen compromise, and restriction of coronary blood flow. It is more frequent in young women, particularly in the peri- and post-partum period and with oral contraceptive use, and in young and middle-aged patients without obvious cardiovascular risk factors .
Coronary intramural hematoma can also occur iatrogenically, as a complication to percutaneous coronary intervention (PCI) . In this patho-physiological scenario, the hematoma begins as a trauma triggered dissection to the media at an atherosclerotic reference segment site, and propagates along the medial plane into a more normal artery segment, but does not re-enter the lumen . The incidence is increasing due to the growing use of interventional diagnostics and treatment of coronary artery disease.
Intravascular ultrasound (IVUS) is an important diagnostic tool, which may provide important morphometric information regarding the magnitude and severity of intramural hematomas. Also, when the decision to treat has been made, IVUS provides precious information regarding vessel size and hematoma length that helps guide PCI treatment and stent selection. Management strategies may range from conservative medical treatment to percutaneous or surgical interventions depending upon the anatomy and extent of the hematoma, compromise of the vessel lumen and the clinical circumstances , and the prognosis varies widely as the numbers of reported cases are limited .
We present three cases of coronary intramural hematomas that originated from different causes, and the important role of IVUS in the diagnostic process and subsequent treatment is highlighted. Based on previous, limited case reports and retrospective analyses, a focused literature review assessing the existing management options and prognosis, is provided in the discussion section.
1.1
Case 1
A 76-year old female with a history of hypertension, smoking and a family history of ischemic heart disease was admitted due to gradual increasing dyspnea over one week. Symptom onset was preceded by a few days with gastroenteritis causing pronounced diarrhea and vomiting. The electrocardiogram (ECG) showed a left bundle branch block (LBBB), and Troponin-I was moderately elevated (5.2 ng/ml). An echocardiogram showed global hypokinesia of the left ventricle with an ejection fraction (EF) of 15%–20%. She was diagnosed as being a “late presenter” with ST segment elevation myocardial infarction (STEMI), and treatment with aspirin, ticagrelor and fondaparinux was started. Also treatment for heart failure due to a poor left ventricular function was initiated.
A subacute coronary angiography (CAG) revealed angiographically normal coronary arteries, apart from a small caliber left main stem (LM), with a diameter less than that of the proximal left anterior descending (LAD) and the left circumflex (CX) ( Fig. 1 , left ). There was Thrombolysis In Myocardial Infarction (TIMI) flow grade 3 in all coronary segments. Subsequently, we performed an IVUS of the LM and LAD, which identified the cause of the luminal narrowing to be a large intramural hematoma starting at the proximal LM and progressing to the mid LAD with a length of approximately 40 mm ( Fig. 2 , row 1 + 2 ). Minimum lumen area (MLA) was 4.8 mm 2 at the LM and 4.1 mm 2 at the LAD. There was no evidence of dissection, neither was there any evidence of atherosclerosis. The patient was hemodynamically stable and asymptomatic, and there were no signs of ongoing ischemia; therefore, a conservative treatment strategy was chosen. The patient was discharged after one week in a stable clinical condition with treatment for heart failure and aspirin.
At one-month follow-up she was well besides mild dyspnea during activity. A repeat echocardiogram revealed sustained severe heart failure with an unchanged EF of 15%–20%. Repeated CAG visualized angiographically normal vessels ( Fig. 1 , right ), and IVUS revealed a near-complete restoration of the intramural hematoma ( Fig. 2 , row 3 + 4 ). The hematoma was completely resolved at the LM, where MLA was estimated to 15.3 mm 2 . A smaller (length 18 mm) and more consolidated intramural hematoma was still visible at the proximal LAD, where MLA was estimated to 8.2 mm 2 . All coronary segments were with TIMI flow grade 3.
1.2
Case 2
A 66-year old female was admitted due to sudden onset of chest pain. Cardiovascular risk factors included active smoking, hypertension and a family history of ischemic heart disease. The ECG showed a chronic LBBB, and Troponin-I was slightly elevated (1.11 ng/ml). The patient was treated with a non-STEMI regimen including aspirin, clopidogrel and low-molecular weight heparin. The echocardiogram disclosed normal wall motions and a preserved EF. A subacute CAG revealed an intermediate lesion in the LAD ( Fig. 3 , A ). However, IVUS confirmed a significant stenosis with an MLA of 2.2 mm 2 , and direct stenting with a drug-eluting stent (DES) (2.75 × 18 mm, Resolute Integrity™) with post-dilation of the proximal stented segment (3.0 mm non- compliant balloon 20 atm.) was performed. After post-dilation, a narrowing of the second diagonal branch (D2) was noted and treated with dilation of a 3.0 mm balloon with a low pressure, which resulted in an angiographically satisfactory result ( Fig. 3 , B ). Final IVUS confirmed a well-apposed and well-expanded stent without visible edge dissections ( Fig. 4 , row 1 + 2 ).
Immediately after mobilization from the operating table to the bed, the patient experienced severe chest pain and the heart monitoring revealed pronounced ST segment elevations. Acute re-CAG confirmed narrowing and irregularities proximally to the index stented segment. A dissection was angiographically suspected ( Fig. 3 , C ). Supplementary IVUS revealed a 10–15 mm long occlusive intramural hematoma proximal to the index stented segment ( Fig. 4 , row 3 + 4 ), without visible dissection flaps, and an additional DES was implanted proximally to the index stented segment (3.5 × 26 mm, 12 atm., Resolute Integrity™). Post-procedure IVUS showed a slight propagation of the hematoma proximally in the LM, but the propagation was without lumen compression ( Fig. 4 , row 5 + 6 ). Hematoma progression extending into the D2 caused TIMI grade flow 0, and an additional DES was implanted (Culotte stenting technique; 2.5 × 12 mm, 12 atm, Resolute Integrity™), including a final “kissing balloon” in LAD/D2 (12 atm). The angiogram ( Fig. 3 , D ) and IVUS revealed further propagation of the hematoma distally in the D2, but due to clinical and hemodynamic stability and TIMI flow grade 3 ( Fig. 3 , E ), no further PCI was performed.
The patient was discharged free of symptoms 3 days after the procedure. A repeat angiogram one week later showed TIMI flow grade 3 in all coronary segments.
1.3
Case 3
A 59-year old male was referred for an elective CAG due to stable angina pectoris. PCI with a DES in the right coronary artery (RCA) had been performed 3 years earlier due to unstable angina. Risk factors included prior smoking, hypertension and hypercholesterolemia. The angiogram and a complementary IVUS showed a severe stenosis proximally in the LAD with a MLA of 2.6 mm 2 , and the lesion was stented directly with a DES (3.5 × 15 mm; 18 atm, Xience V™) ( Fig. 5 , A + B ).
Repeat IVUS showed a slightly under expanded stent, which is why post-dilation with a 3.5 mm non-compliant balloon was performed, and final IVUS confirmed a well-apposed and well-expanded stent without visible edge dissections ( Fig. 6 , row 1 + 2 ). The patient was discharged free of symptoms on the same day as the procedure was performed.
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