Chest pain in a young female

Acute Coronary Syndrome (ACS) is a common diagnosis in the emergency department (ED), the most severe manifestation of which is ST elevation on electrocardiogram (ECG). ST elevation reflects obstruction of flow through the coronary arteries, most commonly due to coronary atherosclerotic plaque rupture. However, alternative causes of coronary obstruction causing ST elevation are possible. Spontaneous coronary artery dissection (SCAD) is an unusual cause of ST elevation in ED patients which providers may encounter in patients without traditional atherosclerosis risk factors. Patients presenting with SCAD as a cause of ST elevation require unique management from traditional ACS. Here we report a case of a 43?year old female presenting with chest pain and unusual ECG findings including accelerated idioventricular rhythm followed by subtle ST segment elevation and resolution of abnormalities. This case illustrates subtle clinical and ECG findings suggestive of SCAD which emergency physicians should consider when evaluating patients for ACS in the absence of traditional clinical presentations. Such considerations may prompt physicians to avoid therapy for coronary plaque rupture which is not indicated in patients with SCAD.     

Current management of spontaneous coronary artery dissection

Introduction: Spontaneous coronary artery dissection (SCAD) remains an infrequent and elusive clinical entity of unknown etiology. However, our knowledge of SCAD has been significantly enriched in recent years. Large and prospective contemporary series have increased the interest in this disease with fewer patients misdiagnosed and a growing number of cases recognized in daily clinical practice. Classically, SCAD was thought to present mainly in young women without traditional atherosclerotic risk factors but, actually, most patients are middle-aged and are not free from coronary risk factors. A high number of associated conditions have been reported. Of these, fibromuscular dysplasia emerges as a major association with intriguing pathophysiological implications.

Areas covered: This review aims to present a contemporary update on SCAD. We concentrate on the clinical scenario, related conditions, practical management and treatment strategies.

Expert commentary: Recognition of SCAD is currently much more frequent and accurate as a result of increased clinical awareness and the widespread use of intravascular imaging techniques. Hopefully, in the near future an improvement in the management of SCAD patients will come not only from empirical evidence but also from dedicated clinical trials.     

A Case of Spontaneous Coronary Artery Dissection: It is Not Always Plaque Rupture

Background

Spontaneous coronary artery dissection (SCAD) is an extremely rare cause of acute coronary syndrome (ACS). Patients may present with a broad spectrum of clinical scenarios, ranging from angina pectoris to myocardial infarction, cardiogenic shock, and sudden death. Standard therapy has not been established; current treatments range from conservative management to percutaneous revascularization or coronary artery bypass surgery.

Objective

SCAD greatly mimics ACS, and this diagnosis should be considered when evaluating young patients who present with ACS with or without classical risk factors for coronary artery disease.

Case Report

We report a case of a 45-year old man who presented with chest pain typical of ACS. He had no risk factors except for a smoking history of 2.5 pack-years. Once the clinical findings suggested acute inferolateral myocardial infarction, the patient underwent emergent cardiac catheterization, which revealed left anterior descending coronary artery dissection. This in itself is not a common cause of inferolateral ST elevation changes on electrocardiogram.

Conclusion

This case highlights the fact that although SCAD is a rare entity, it is increasingly being recognized as a significant cause of ACS. Urgent angiography should be considered if SCAD is suspected, because early diagnosis and appropriate management significantly improve the outcome in these patients.     

Spontaneous Coronary Artery Dissection in a 27-Year-Old Woman

Background

Spontaneous coronary artery dissection (SCAD) causes acute coronary syndromes or sudden death in young patients who are often lacking classic coronary disease risk factors. Systemic inflammatory and connective tissue diseases have been suggested as risk factors for SCAD.

Objective

To review the risk factors, diagnosis, and management of this uncommon but life-threatening disease.

Case Report

We report a case of a 27-year-old woman with a history of an ill-defined inflammatory arthropathy who presented with an acute ST-elevation myocardial infarction. SCAD was diagnosed by coronary angiography. Percutaneous coronary intervention was attempted but was unsuccessful. The patient recovered uneventfully with medical management and was ultimately diagnosed with systemic lupus erythematosus.

Conclusions

SCAD is a rare but important cause of acute coronary syndromes and sudden death. It commonly occurs in young women. Although pregnancy is the most well-established risk factor, systemic inflammatory and connective tissue diseases have also been suggested as risk factors.     

Spontaneous coronary artery dissection: a review of complications and management strategies

Introduction: Spontaneous coronary artery dissection (SCAD) accounts for up to 4% of acute coronary syndromes (ACS) on coronary angiography and predominantly affects women (>90%). SCAD most often occurs in patients with few or no conventional cardiovascular risk factors. This condition remains underdiagnosed due to a lack of awareness among health-care providers and misdiagnosis on coronary angiography.

Areas covered: In this review, authors aim to summarize contemporary data on the etiology, morbidity and mortality risks, as well as management strategies in order to raise awareness.

Expert opinion: The last decade of extensive research revealed that SCAD is not as rare as previously thought and needs to be in the differential diagnosis of all MI patients, especially in young to middle-aged women. Predisposing factors, such as fibromuscular dysplasia, and potential triggers like physical or emotional stresses, are frequently linked. Coronary angiography is the first-line diagnostic tool and should be performed meticulously because of more fragile vessel architecture to avoid iatrogenic dissections. If angiographic findings are uncertain, the use of intravascular imaging may be required. Conservative therapy is favored over revascularization unless patients have high-risk features. Recurrent cardiovascular events post-SCAD are frequent, and patients should be closely monitored after a SCAD event.     

Successful Wallstent implantation for extensive iatrogenic renal artery dissection in a patient with fibromuscular dysplasia.

PURPOSE: To describe a case of renal artery stenosis with fibromuscular dysplasia (FMD) and extensive iatrogenic dissection treated with Wallstent implantation. METHODS AND RESULTS: An 83-year-old woman with a history of coronary artery disease and hypertension presented at another facility with exertional angina and poorly controlled hypertension. Renal arteriography uncovered a critical right renal artery stenosis with severe FMD. However, angioplasty resulted in extensive dissection of the renal artery, for which the patient was referred to our institution. The renal artery was recanalized via the left brachial approach with restoration of flow using a Wallstent and a Palmaz stent. The patient's blood pressure was controllable after this procedure, and follow-up duplex imaging with flow velocities at 6 months showed patent right renal artery stents. CONCLUSIONS: Owing to its length and flexibility, the Wallstent endoprosthesis was a useful treatment modality in this case of extensive renal artery dissection.     

Spontaneous coronary artery dissection: etiology and recurrence

Introduction: Spontaneous coronary artery dissection (SCAD) is an increasingly appreciated cause of acute myocardial infarction (AMI) and sudden cardiac death most often affecting young to middle-aged women with few conventional cardiovascular risk factors.

Areas covered: A literature search was performed using MedLine, PubMed, and Google Scholar (dating to 04/30/2019). Authors review the key clinical features of SCAD and highlight what is known regarding its pathophysiology and associated factors. The relationship between SCAD and other systemic vasculopathies, notably fibromuscular dysplasia (FMD) is also discussed. Authors also mention the management of acute SCAD along with considerations for long term follow-up such as chest pain syndrome, extracoronary vasculopathy screening, and recurrent SCAD.

Expert opinion: Our understanding regarding the association of SCAD and other arteriopathies such as FMD is anticipated to grow. In addition, progress is likely to be made in our efforts to predict recurrent SCAD risk and define potential preventative strategies, possibly through the incorporation of adjunctive imaging.     

Apical hypertrophic cardiomyopathy: a case of slow flow in lad and malign ventricular arrhythmia

The coronary slow flow phenomenon is an angiographic finding characterized by delayed distal vessel opacification in the absence of epicardial coronary artery disease. Patients often present with acute coronary syndrome. Histopathologic studies have revealed the existence of fibromuscular hyperplasia and myofibrilar hypertrophy. Apical hypertrophic cardiomyopathy is a benign progressive form of hypertrophic cardiomyopathy, that is rarely observed in western communities. It remains commonly asymptomatic until advanced ages. Syncope, arrhythmia or sudden death may be the first symptom. We report a case of slow coronary arterial flow in a 71-year-old male patient with apical hypertrophic cardiomyopathy who experienced chest pain and sudden cardiac arrest due to ventricular arrhythmia.     

Spontaneous dissection of proximal left main coronary artery in a healthy adolescent presenting with syncope: A case report

BACKGROUNDSpontaneous coronary artery dissection (SCAD) is a frequent cause of acute coronary syndrome in young to middle-aged women with few or no traditional cardiovascular risk factors. Chest pain is the most frequently described presenting symptom, but syncope is extremely rare. Herein, we report on a 16-year-old girl who presented with an episode of syncope occurring during a race. Despite significantly elevated troponin level, the diagnosis of the left main coronary artery SCAD with cardiogenic shock was delayed. CASE SUMMARYA 16-year-old girl presented with an episode of syncope. Myocardial injury markers were positive. Echocardiography showed a mildly reduced left ventricular ejection fraction (50%). Although initially stable, she later experienced recurrent chest pain accompanying precordial ST segment elevation with dynamic changes and developed cardiogenic shock, necessitating emergent revascularization. Coronary angiography demonstrated almost total occlusion at the ostium and proximal segment of the left main trunk coronary artery (LMT). Intravascular ultrasound confirmed a false lumen with prominent dissection in the LMT. Percutaneous coronary intervention assisted by intra-aortic balloon pump was conducted in the LMT. A 3.5 mm × 24 mm everolimus-eluting stent was deployed to the focal lesions of the LMT. A postprocedural electrocardiogram showed alleviation of the precordial ST-segment elevation. The diagnosis of SCAD was confirmed. Transthoracic echocardiography showed an improved left ventricular ejection fraction (57%). The patient was asymptomatic during the 24-mo. follow-up period.CONCLUSIONSCAD should always be considered in the differential diagnosis of acute coronary syndrome presentations in low-risk patients, regardless of age.     

Eosinophilic granulomatosis with polyangiitis presenting with repetitive acute coronary syndrome,refractory coronary vasospasm,and spontaneous coronary dissection: a case report

Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of eosinophilic vasculitis that is mainly limited to small- and medium-sized arteries. Cardiac involvement is the leading cause of death in patients with EGPA. Spontaneous coronary artery dissection (SCAD) is an important cause of acute coronary syndrome in middle-aged women with no or few traditional cardiovascular risk factors. EGPA manifesting as repetitive acute coronary syndrome and SCAD has not been reported. A 45-year-old woman presented with recurrent chest pain and cardiogenic shock associated with coronary vasospasm refractory to common vasodilators. Coronary angiography showed SCAD at the proximal right coronary artery. Blood tests showed significant eosinophilia. In addition to sinusitis as shown by nasal computed tomography and abnormal nerve conduction velocity, the diagnosis of EGPA was made and immunosuppression commenced. During a 20-month follow-up, the patient remained free from symptoms and adverse cardiovascular events. EGPA can involve coronary arteries and may rarely manifest as SCAD or vasospasm. We herein review the mechanism underlying coronary involvement of EGPA and emphasize special clues for its detection. Early recognition and initiation of immunosuppression therapy are important.     

Spontaneous coronary dissection should not be ignored in patients with chest pain in autosomal dominant polycystic kidney disease: A case report

BACKGROUNDWhen autosomal dominant polycystic kidney disease (ADPKD) presents with acute coronary syndrome (ACS), the possibility of spontaneous coronary artery dissection (SCAD) should be highly considered. In some cases, SCAD is considered an extrarenal manifestation of ADPKD depending on the pathological characteristics of the unstable arterial wall in ADPKD. CASE SUMMARYHere, we report a 46-year-old female patient with ADPKD who presented with ACS. Coronary angiography revealed no definite signs of dissection, while intravascular ultrasound revealed a proximal to distal dissection of the left circumflex. After a careful conservative medication treatment, the patient exhibited favorable prognosis. CONCLUSIONIn cases of ADPKD co-existing with ACS, differential diagnosis of SCAD should be considered. Moreover, when no clear dissection is found on coronary angiography, IVUS should be performed to prevent missed diagnosis.     

Successful revascularization with percutaneous coronary intervention using a combination of the subintimal transcatheter withdrawal technique and coronary artery fenestration for spontaneous coronary artery dissection

The combination of the STRAW technique and coronary artery fenestration using a cutting balloon could be effective in SCAD patients, especially with dissection to the distal end of the coronary artery.     

Recognizing and managing fibromuscular dysplasia

Fibromuscular dysplasia typically occurs in young women and most commonly presents with hypertension, transient ischemic attack, stroke, or an asymptomatic cervical bruit. The disease is nonatherosclerotic and noninflammatory and most often affects the renal and carotid arteries, although almost any artery can be involved. On angiography, affected blood vessels characteristically resemble a string of beads in the most common type of fibromuscular dysplasia, medial fibroplasia. Patients with renal artery stenosis and hypertension or renal impairment should be treated with percutaneous transluminal angioplasty without a stent. Patients with fibromuscular dysplasia of the internal carotid artery should also be treated with angioplasty if they develop focal neurologic symptoms such as a transient ischemic attack or stroke.     

Rapidly and Accurately Detecting Significant Coronary Artery Stenosis in Patients with Suspected Stable Coronary Artery Disease and Normal Segmental Wall Motion by Speckle Tracking Automated Functional Imaging

This study was performed to explore the value of a machine with built-in speckle tracking automated functional imaging (AFI) in predicting the severity of coronary artery lesions in patients with suspected stable coronary artery disease (SCAD) with normal wall motion. One hundred forty-three consecutive patients with clinically suspected SCAD with normal wall motion were included. Multiple parameters, including global longitudinal peak strain, post-systolic index (PSI) and peak strain dispersion, were automatically analyzed with AFI. Territorial longitudinal strain (TLS) and territorial PSI (TPSI) were also calculated. Receiver operating characteristic curve analyses revealed that global longitudinal peak strain and PSI were superior to other parameters in detecting left main or three-vessel SCAD. A combination of TLS and TPSI could improve diagnostic performance in identifying significant stenotic left anterior descending and right coronary arteries. In conclusion, 2-dimensional speckle tracking AFI can rapidly provide multiple parameters for detecting significant coronary artery stenosis with high accuracy in patients suspected of having SCAD with normal wall motion.     

PCSK9 and atherosclerosis burden in the coronary arteries of patients undergoing coronary angiography

AimsTo investigate the association between plasma proprotein convertase subtilisin/kexin type 9 (PCSK9) concentrations, current acute coronary syndrome (ACS), coronary artery disease (CAD) presence, severity and extension and the burden of coronary calcifications in patients with suspected CAD.Methods and resultsOne hundred and one patients, with or without current ACS, were recruited for this cross-sectional study. CAD presence was defined based on either the presence or absence of at least one significant (≥50%) CAD lesion (SCAD). CAD severity was classified according to the absence of coronary lesions, the presence of non-significant (<50%) CAD (MCAD) or SCAD in at least one major coronary artery. Patients with one, two or three significantly diseased major coronary arteries were defined as 1-SCAD, 2-SCAD and 3-SCAD, respectively. The cumulative length of SCAD lesions and the amount of calcifications in coronary arteries were estimated. Plasma PCSK9 concentrations were higher in patients with SCAD as compared to those without (p = .012). A significant increase in plasma PCSK9 concentrations was observed with greater CAD severity (p = .042). Higher plasma PCSK9 concentrations were found in 3-SCAD patients as compared to either 2-SCAD or 1-SCAD (p < .001). PCSK9 increased with the cumulative length of SCAD lesions and the burden of calcifications (p < .05 for both comparisons). Multivariable adjustment abolished the association between PCSK9 and either CAD presence or severity, but not the association between PCSK9 and the number of significantly diseased vessels, SCAD lesion length and the burden of coronary calcifications. ACS was associated with a borderline significant increase of plasma PCSK9 concentrations among patients not taking statins (p = .05).ConclusionCirculating PCSK9 concentrations discriminate patients with greater coronary atherosclerotic lesion extension and calcification, and are increased in patients with current ACS.     

Spontaneous dissection in the left main coronary artery

We present two cases of spontaneous left main stem coronary artery dissection. One was underdiagnosed with suboptimal percutaneous coronary artery intervention followed by acute vessel occlusion again during the hospitalization. The other one was identified and confirmed by intravascular ultrasound, followed by conservative medical treatment, with completed healing of SCAD during two-month follow up.     

Renovascular hypertension

Renal artery disease is the most common cause of potentially curable secondary hypertension, with atherosclerosis as the major cause of renal artery stenosis. Fibromuscular dysplasia is a less common cause of renal artery stenosis and is most frequently observed in premenopausal women. Renal artery stenosis is likely to be underappreciated and is more common in patients with other vascular disease (e.g., coronary or peripheral arterial disease). The diagnosis of renal artery stenosis requires a high clinical index of suspicion as well as an appropriate imaging strategy, with currently effective diagnostic modalities including magnetic resonance imaging, computed tomography and renal artery duplex ultrasonography. The current treatment of choice for atherosclerotic renal artery stenosis is balloon angioplasty and secondary stenting, whereas angioplasty alone is the treatment for renal artery stenosis secondary to fibromuscular dysplasia. Expected outcomes following revascularization include improved blood pressure control and possibly renal preservation. Ongoing studies will hopefully identify patient characteristics that will achieve the most benefit from percutaneous revascularization as well as the impact of percutaneous revascularization with drug-eluting stents and embolic protection devices.     

Renovascular hypertension

Renal artery disease is the most common cause of potentially curable secondary hypertension, with atherosclerosis as the major cause of renal artery stenosis. Fibromuscular dysplasia is a less common cause of renal artery stenosis and is most frequently observed in premenopausal women. Renal artery stenosis is likely to be underappreciated and is more common in patients with other vascular disease (e.g., coronary or peripheral arterial disease). The diagnosis of renal artery stenosis requires a high clinical index of suspicion as well as an appropriate imaging strategy, with currently effective diagnostic modalities including magnetic resonance imaging, computed tomography and renal artery duplex ultrasonography. The current treatment of choice for atherosclerotic renal artery stenosis is balloon angioplasty and secondary stenting, whereas angioplasty alone is the treatment for renal artery stenosis secondary to fibromuscular dysplasia. Expected outcomes following revascularization include improved blood pressure control and possibly renal preservation. Ongoing studies will hopefully identify patient characteristics that will achieve the most benefit from percutaneous revascularization as well as the impact of percutaneous revascularization with drug-eluting stents and embolic protection devices.     

A refractory hypertensive patient with fibromuscular dysplasia and multicystic dysplastic kidney

The case of a 16-year-old male patient with left renal artery stenosis due to fibromuscular dysplasia (FMD) and an atrophic kidney due to a right multicystic dysplastic kidney (MCDK) who presented with refractory hypertension is reported. On continuous Doppler imaging, the peak systolic velocity (PSV) at the stenotic site of the left renal artery was 404 cm/s. The FMD formed a “string-of-beads” appearance on computed tomographic angiography (CTA) and renal artery angiography. Percutaneous transluminal renal angioplasty (PTRA) with a balloon catheter was performed, after which the left renal artery returned to near normal, and his blood pressure decreased to within the normal range. In this case, the main cause of refractory hypertension was considered to have been renal artery stenosis due to left FMD.     

Percutaneous closure of a fistula from the left circumflex coronary artery to the coronary sinus in an infant

Coronary artery fistula is an abnormal direct connection between the coronary artery and any of the four chambers of the heart or great vessels. A fistula from the left circumflex coronary artery to the coronary sinus is a relatively rare situation. We report a case of 12-month-old infant with coronary artery fistula from the left circumflex coronary artery to the coronary sinus that was identified incidentally. The N-terminal pro-brain natriuretic peptide level was elevated. Additionally, the proximal segment of the left circumflex coronary artery was dilated. On the basis of these findings, percutaneous closure of the fistula was performed with a vascular plug. This procedure achieved no residual flow and good hemodynamics were observed during follow-up.