Compromiso cardiovascular en pacientes con síndrome inflamatorio pediátrico multisistémico, asociado a infección por SARS-CoV-2 / Cardiovascular involvement in pediatric patients with SARS-CoV-2 associated to multisystemic inflammatory syndrome

INTRODUCCION: El Síndrome Inflamatorio Pediátrico Multisistémico (PIMS) ha emergido como una nueva enfermedad en niños, secundaria a infección por SARSCoV-2. Se caracteriza por presentar compromiso multiorgánico con parámetros inflamatorios elevados y manifestaciones clínicas graves, siendo el corazón el órgano más severamente comprometido. OBJETIVO: Describir las características clínicas y de laboratorio de 23 pacientes con diagnóstico de PIMS con compromiso cardiovascular hospitalizados en un centro único. MÉTODO: Se efectuó un estudio retrospectivo analizando los hallazgos clínicos y de laboratorio junto a las manifestaciones cardiovasculares que presentaron estos pacientes. RESULTADOS: 23/29 pacientes con PIMS (78%) presentaron manifestaciones digestivas y mucocutáneas. Las manifestaciones cardiovasculares fueron: Síndrome Kawasaki y "Kawasaki like" sin compromiso coronario en 15/23 (65%) y con compromiso coronario en 3 (13%). Shock en 9 pacientes (39%), injuria miocárdica- miocarditis en 8 (35%) y derrame pericárdico en 13 (56%). Trastornos del ritmo cardíaco se observaron en 6 pacientes (26%). La terapia más utilizada fue inmunoglobulina y corticoides. 18 /23 requirieron manejo en unidades de intermedio y/o intensivo. Un 70% de los pacientes se recuperó del compromiso cardiovascular antes del alta. CONCLUSIÓN: El compromiso cardiovascular en PIMS es la complicación más frecuente de esta enfermedad, que se acompaña de manifestaciones inmunológicas y hematológicas graves lo que hace necesario un tratamiento multidisciplinario para un mejor manejo de estos pacientes.

INTRODUCTION: Pediatric Multisystemic Inflammatory Syndrome (PIMS) has emerged as a new disease in children, secondary to SARSCoV-2 infection. It is characterized by multi-organ involvement with elevated inflammatory parameters and severe clinical manifestations, the heart being the organ most severely involved. OBJETIVE: to describe the clinical and laboratory characteristics of 23 patients diagnosed with PIMS with cardiovascular involvement hospitalized in a single center. METHOD: We conducted a retrospective study in which we analyzed the clinical and laboratory findings along with the cardiovascular manifestations presented by these patients. Results: 23/29 patients with PIMS and cardiovascular involvement were selected, 78% had digestive and mucocutaneous manifestations. Cardiovascular manifestations consisted of KawasakiKawasaki like syndrome without coronary involvement in 15/23 (65%) and coronary involvement in 3 (13%). Nine patients developed shock (39%), 8 (35%) myocardial injury in and 13 (56%) pericardial effusion.. Heart rhythm disorders were observed in 6 patients (26%). The main therapy was immunoglobulin and corticosteroids. 18 /23 required management in intermediate and/or intensive care unit. 70% of patients recovered from cardiovascular involvement before discharge. CONCLUSION: Cardiovascular involvement in PIMS is the most frequent complication of this disease, but it is associated with severe immunological and hematological manifestations, which makes necessary a multidisciplinary treatment for a better management

Frequency of risk factors in patients of acute coronary syndrome due to coronary ectasia.

BACKGROUND: Coronary artery ectasia is a relatively common entity characterized by inappropriate dilatation of the coronary vasculature. In some cases of acute coronary syndrome without obstructive coronary lesions, coronary ectasia is the sole cause. The exact mechanism of its development is unknown but evidence suggests a combination of genetic predisposition, common risk factors for coronary artery disease, and abnormal vessel wall metabolism. As there are few data regarding the pattern of coronary risk factors in patients with coronary ectasia, the objective of the study was to determine the frequency and distribution of coronary risk factors in patients with acute coronary syndrome solely due to coronary ectasia. METHODS: The study included 155 patients over a period of 6 months, with coronary angiographic evidence of coronary ectasia as the sole cause of acute coronary syndrome. There were 79 (51%) men and 76 (49%) women with a mean age 51.92 ± 7.83 years; 73 (47.10%) were aged 20-50 years and 82 (52.90%) were 51-80 years of age. The frequencies of coronary risk factors were stratified according to sex and the two age groups. RESULTS: Seventy-one patients (45.80%) had diabetes mellitus, 83 (53.54%) had hypertension, 55 (35.48%) were smokers, 46 (29.68%) had dyslipidemia, and 47 (30.3%) were obese. CONCLUSION: Hypertension is the leading coronary risk factors in patients with acute coronary syndrome solely due to coronary ectasia, followed by diabetes mellitus and smoking.

Hypomethylation of C1q/tumor necrosis factor-related protein-1 promoter region in whole blood and risks for coronary artery aneurysms in Kawasaki disease.

BACKGROUND: Kawasaki disease (KD) is characterized as a self-limited systemic vasculitis. C1q/tumor necrosis factor-related protein-1 (CTRP1) had been associated with the occurrence of vasculitis in KD. Methylation at the promoter region of certain genes was reported to be involved in the development process of KD. This study aims to investigate the methylation levels of CTRP1 in KD, as well as, its potential to predict coronary artery aneurysms (CAAs). METHODS: 31 patients with KD and 14 healthy controls (HCs) were recruited into this study. The KD group was further divided into KD with CAA (KD-CAAs) group and KD without NCAAs (KD-NCAAs) group. Methylation levels of CpG sites were determined by MethylTarget sequencing, a method that uses multiple targeted CpG methylation analysis. RESULTS: The methylation levels of CTRP1 promoter region in the KD group were lower than that in the HC group at all predicted CpG sites, especially at sites 34, 51, 69, 79, 176 and 206. Compared with KD-CAAs group, the methylation levels of almost every CpG sites of CTRP1 were increased in the KD-NCAAs group, with site 69 and 154 found to be strongly related to the occurrence of CAAs. CONCLUSIONS: The difference in methylation levels of CTRP1 promoter may be involved in the development process of KD, and may be a potential predictive marker for the occurrence of CAAs.

Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms.

Yilmazer MM, Özdemir R, Mese T, Küçük M, Öner T, Devrim I, Bayram N, Güven B, Tavli V. Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms. Turk J Pediatr 2019; 61: 648-656. Prompt diagnosis and the administration of intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery abnormalities (CAA) in Kawasaki Disease (KD). The resistance to treatment and development of the coronary sequelae remain the most important problems in KD. We aimed to determine the predicting factors of nonresponse to initial IVIG therapy and to analyze the cases who had giant coronary aneurysms. A total of 120 KD cases, including 61 children fulfilling the criteria for KD and 59 with incomplete KD were enrolled into this study. Demographic, laboratory, clinical, echocardiographic characteristics, and treatment regimens were reviewed, retrospectively. The median age of the patients was 33.5 months (range: 3-168 months). Coronary artery aneurysms were detected in 35 patients (29%) at the time of diagnosis. Twenty-eight patients had coronary aneurysms small or medium in size, one had a large, and seven had giant coronary aneurysms. CAA persisted in 8 cases in the follow-up, all of which were large or giant aneurysms. A ten month-old girl with a giant coronary aneurysm was referred to coronary bypass surgery in the subacute phase of follow-up, due to myocardial ischemia. Eighteen patients were unresponsive to the initial IVIG therapy (%15), of whom 10 were diagnosed as cKD and 8 were iKD. Patients who did not respond to initial IVIG therapy, had higher white blood cell (WBC) count, higher C-reactive protein (CRP) and lower albumin levels than those who did (P < 0.05). In univarite analysis; CRP, WBC and albumin were found to be significant predictors of nonresponse to initial IVIG therapy, while a stepwise multiple linear regression analysis showed that WBC count and albumin levels were significantly correlated with nonresponse to initial treatment with IVIG. Our study showed that WBC count and albumin levels might be used as predictors of nonresponse to the IVIG therapy in Turkish children with KD.

Conditions and Factors Associated With Spontaneous Coronary Artery Dissection (from a National Population-Based Cohort Study).

The pathophysiology of spontaneous coronary artery dissection (SCAD) is heterogeneous, associated with systemic arteriopathies and inflammatory diseases, and often compounded by environmental precipitants, genetics, or stressors. However, the frequency of these associated conditions with SCAD on a population level remains unknown. Therefore, the objective of this analysis was to evaluate heterogeneous phenotypes of SCAD in the United States using data from the Nationwide Inpatient Sample collected from January 1, 2004, to September 31, 2015. Among 66,360 patients diagnosed with SCAD, the mean age was 63.1 ± 13.2 years and 44.2% were women. A total of 3,415 (5.14%) had depression, 670 (1.0%) had rheumatoid arthritis, 640 (0.96%) had anxiety, 545 (0.82%) had a migraine disorder, 440 (0.66%) used steroids, 385 (0.58%) had malignant hypertension, 280 (0.42%) had systemic lupus erythematosus, 250 (0.38%) had cocaine abuse, 215 (0.32%) had hypertensive heart or renal disease, 130 (0.19%) had coronary spasm, 105 (0.16%) had fibromuscular dysplasia, 85 (0.13%) had Crohn's disease, 75 (0.11%) had celiac disease, 60 (0.09%) had adult autosomal dominant polycystic kidney disease, 60 (0.09%) had hormone replacement therapy, 55 (0.08%) had sarcoidosis, 55 (0.08%) had amphetamine abuse, 15 (0.02%) had granulomatosis polyangiitis, 10 (0.02%) had α1-antitrypsin deficiency, 10 (0.02%) had Marfan syndrome, 10 (0.02%) had Ehlers-Danlos syndrome, 10 (0.02%) had Kawasaki disease, 10 (0.02%) had polyarteritis nodosa, and 5 (0.01%) had multiparity. In conclusion, most cases of SCAD had no apparent concomitant arteriopathy, inflammatory disorder, or evident risk factor.

Management of Coronary Artery Aneurysms.

Aneurysmal dilation of coronary arteries is observed in up to 5% of patients undergoing coronary angiography. Due to their poorly elucidated underlying mechanisms, their variable presentations, and the lack of largescale outcome data on their various treatment modalities, coronary artery aneurysms and coronary ectasia pose a challenge to the managing clinician. This paper aims to provide a succinct review of aneurysmal coronary disease, with a special emphasis on the challenges associated with its interventional treatment.

Manifestaciones cardiacas en la etapa aguda de la enfermedad de Kawasaki en un hospital pediátrico de tercer nivel en la Ciudad de México / Cardiac manifestations in the acute phase of Kawasaki disease in a third level children's hospital in Mexico City

Resumen Objetivo: Describir las manifestaciones cardiacas en la etapa aguda de la enfermedad de Kawasaki en pacientes atendidos en un hospital de tercer nivel de la Ciudad de México, México. Métodos: Estudio retrospectivo, descriptivo en pacientes con diagnóstico de enfermedad de Kawasaki de agosto de 1995 a diciembre del 2016 en el Instituto Nacional de Pediatría, México. Se estudio la demografía de los pacientes, características clínicas, tratamiento empleado y desarrollo de complicaciones cardiacas en la etapa aguda de la enfermedad. Resultados: Se estudiaron 508 casos de enfermedad de Kawasaki. La edad media al diagnóstico fue de 37.64 ± 35.56 meses. Predominio de pacientes masculinos del 65.4%, con una relación masculino/femenino de 1.88:1. La mayoría de los casos (79.2%) tuvo una presentación completa. La gammaglobulina intravenosa fue administrada en 92.4% de los casos. Veintiocho pacientes (5.5%) desarrollaron arritmias, se presentaron cambios en el segmento ST en 29 pacientes (5.6%) y 5 pacientes desarrollaron isquemia miocárdica. En el ecocardiograma inicial, 51 pacientes (9.9%) presentaron datos de miocarditis, 72 pacien- tes (14%) datos de pericarditis y 77 casos tuvieron derrame pericárdico (15%). Se detectaron alteraciones en las arterias coronarias en 169 casos (32.9%). Cuatro pacientes fallecieron en la etapa aguda de la enfermedad por complicaciones cardiacas de la enfermedad de Kawasaki. Conclusiones: En México cada vez existen más casos de enfermedad de Kawasaki con un alto porcentaje de manifestaciones cardiacas al diagnóstico. Se requiere de un mayor conocimiento de la enfermedad en México, para poder establecer cuál es la evolución cardiológica de los pacientes en el país.

Abstract Objectives: To describe the cardiac manifestations in the acute phase of patients with Kawasaki disease treated in a third level Children's hospital in Mexico City, Mexico. Methods: A cross-sectional study was conducted in patients with a diagnosis of Kawasaki disease treated in this hospital from August 1995 to December 2016. Information included patient demographics, clinical features, treatment used, electrocardiographic findings, extra-coronary echocardiographic findings, and the development of coronary artery aneurysms in the acute phase of the disease. Results: The study included 508 cases of Kawasaki disease, with a mean age at diagnosis of 37.64 ± 35.56 months (range from 2 to 200 months). Almost two-thirds (65.4%) of the patients were male, with a male/female ratio of 1.88:1. Complete Kawasaki disease was diagnosed in 79.2% of cases. Almost all cases (92.4%) received intravenous immunoglobulin. Twenty-eight patients (5.5%) developed arrhythmias, ST changes developed in 29 patients (5.6%), and 5 patients presented with ischaemic changes. In the initial echocardiographic evaluation, 51 patients (9.9%) were diagnosed with myocar- ditis, 72 patients (14.0%) with pericarditis and 77 cases (15.0%) developed pericardial effusion. Coronary artery anomalies were detected in 169 cases (32.9%). 32 cases were diagnosed as giant coronary aneurysms. Four patients died from cardiac complications in the acute phase of the disease. Conclusions: There has been an increase in the diagnosis of Kawasaki disease in Mexico. They presented with more cardiac complications than reported in literature. An increased knowledge of Kawasaki disease is required in Mexico in order to establish the cardiac outcomes of this group of patients.

Coronary Artery Aneurysms in Kawasaki Disease: Risk Factors for Progressive Disease and Adverse Cardiac Events in the US Population.

BACKGROUND: The natural history of coronary artery aneurysms (CAA) after intravenous immunoglobulin (IVIG) treatment in the United States is not well described. We describe the natural history of CAA in US Kawasaki disease (KD) patients and identify factors associated with major adverse cardiac events (MACE) and CAA regression. METHODS AND RESULTS: We evaluated all KD patients with CAA at 2 centers from 1979 to 2014. Factors associated with CAA regression, maximum CA z-score over time (zMax), and MACE were analyzed. We performed a matched analysis of treatment effect on likelihood of CAA regression. Of 2860 KD patients, 500 (17%) had CAA, including 90 with CAA z-score >10. Most (91%) received IVIG within 10 days of illness, 32% received >1 IVIG, and 27% received adjunctive anti-inflammatory medications. CAA regression occurred in 75%. Lack of CAA regression and higher CAA zMax were associated with earlier era, larger CAA z-score at diagnosis, and bilateral CAA in univariate and multivariable analyses. MACE occurred in 24 (5%) patients and was associated with higher CAA z-score at diagnosis and lack of IVIG treatment. In a subset of patients (n=132) matched by age at KD and baseline CAA z-score, those receiving IVIG plus adjunctive medication had a CAA regression rate of 91% compared with 68% for the 3 other groups (IVIG alone, IVIG ≥2 doses, or IVIG ≥2 doses plus adjunctive medication). CONCLUSIONS: CAA regression occurred in 75% of patients. CAA z-score at diagnosis was highly predictive of outcomes, which may be improved by early IVIG treatment and adjunctive therapies.

Risk factors and outcomes in patients with ectatic infarct-related artery who underwent primary percutaneous coronary intervention after ST elevated myocardial infarction.

OBJECTIVE: We aimed to assess the risk factors for coronary artery ectasia (CAE) as infarct-related artery (IRA) and short-term and 1 year outcomes. BACKGROUNDS: CAE in patients with ST elevated myocardial infarction (STEMI) is a rare condition with a limited knowledge about the risk factors associated with coronary artery ectasia in STEMI and prognosis after primary percutaneous coronary interventions. METHODS: Retrospectively, 1655 patients with STEMI who were undergone coronary angiography were included in this study. Patients were divided into two groups according to their coronary anatomy as ectasia and control groups. Demographic features, angiographic results, and clinical events were compared. Multivariate analysis was performed to assess the association of the features with CAE in STEMI. RESULTS: In total, 1655 patients (99 patients in CAE group vs 1556 patients in control group) were analyzed. Hypertension and smoking were significantly higher in CAE group. No-reflow rates were significantly higher (13.1% vs 5.4%, p = 0.004) in CAE group. In-hospital mortality rates were similar between the groups. Difference in revascularization rates (8.1% vs 9.6%, p = 0.39) and death in 1 year (6.1% vs 4.9%, p = 0.37) were also nonsignificant between the groups. According to results of the multivariate analysis, hypertension (Odds ratio (OR): 1.71 (1.14-2.58), p = 0.01) and smoking (OR: 1.98 (1.32-2.99), p = 0.001) remained significantly associated with coronary ectasia. CONCLUSION: In conclusion, despite being higher no-reflow rates, short-term and 1 year survival and revascularization rates were similar between the groups. Additionally, hypertension and smoking were associated with CAE as IRA. © 2016 Wiley Periodicals, Inc.

Multiple giant coronary artery aneurysms: a case report.

BACKGROUND: Coronary artery aneurysm is defined as a localized area of dilatation exceeding the diameter of the adjacent normal arterial segment by 50%. Giant aneurysms are those aneurysms that measure greater than 2cm in diameter. There have been many pathologic diseases, including atherosclerosis, that have been implicated in the development of coronary artery aneurysms. MATERIALS AND METHODS: We report a case of a 61-year-old African American male with multiple comorbidities including hypertension, congestive heart failure, abdominal aortic aneurysm, and bilateral iliac aneurysms, who was admitted to our hospital with exacerbation of congestive heart failure. Less than 2weeks after admission, the patient suffered cardiac arrest while receiving dialysis and was unresponsive to resuscitative measures. FINDINGS: Autopsy was performed and revealed significant cardiomyopathy and giant coronary artery aneurysms involving the left anterior descending, left circumflex, and right coronary arteries. Both ventricles showed hypertrophy and dilation with multifocal areas of chronic myocardial scarring. CONCLUSIONS: Coronary artery aneurysms and giant coronary artery aneurysms are an uncommon. As there are few reported cases in the literature, the cause, detection, and treatment of this disease are still largely unknown.

Coronary artery aneurysms associated with ascending aortic aneurysms and abdominal aortic aneurysms: pathophysiologic implications.

BACKGROUND: Coronary artery aneurysms (CAA) are seen in 1-5% of angiograms. Aneurysmal coronary disease has been thought to be a variant of atherosclerotic coronary artery disease (CAD) in most patients, but this has not been systematically studied. METHODS: To better understand the pathophysiology of CAA, we reviewed the cardiac catheterization films of 403 patients with ascending thoracic aortic aneurysms and 74 patients with abdominal aortic aneurysms (AAA) who underwent surgery for their aortic aneurysms at our institution. Coronary aneurysms had diameters 1.5-fold that of a reference segment. RESULTS: The incidences of CAA in patients with ascending aneurysms and AAA were 17% and 16% respectively (P = 0.92). CAAs in the ascending group were larger (mean diameter 5.9 vs. 5.0 mm, P = 0.12) with larger reference vessel size (3.1 vs. 2.6 mm, P = 0.03). CAAs in the patients with ascending aneurysms were less likely to be CAD-associated within the same vessel (12% vs. 75%, P < 0.001). This difference remained significant after controlling for the presence of generalized CAD. No other differences in CAAs between the two groups were found. Within the ascending aneurysm group, the only clinical variable independently associated with CAA was bicuspid aortic valve (OR 0.47, 95% confidence interval 0.25-0.89, P = 0.02). The majority of patients with CAA in the ascending aortic aneurysm population did not have CAD or any other previously identified cause of CAA. CONCLUSIONS: There is a high incidence of CAA in patients with aortic aneurysms. In patients with ascending aortic aneurysms there is likely a mechanism distinct from CAD that causes CAAs.

Nationwide survey of coronary aneurysms with diameter >6 mm in Kawasaki disease in Korea.

BACKGROUND: We investigated the course of coronary aneurysm with diameter > 6 mm in Kawasaki disease (KD), as well as related therapeutic trends and prognosis in Korea. METHODS: A nationwide questionnaire survey was carried out in 77 hospitals, to investigate, retrospectively, patients with KD who had coronary aneurysms with a diameter > 6 mm between 1990 and 2011. RESULTS: The median age of onset was 3 years (range, 2 months-16 years) in a total of 239 patients. During the acute stage of KD, most patients received i.v. immunoglobulins and aspirin. In addition, 27 patients received steroid therapy. In the current study, the mean coronary aneurysm size was 8.7 ± 3.2 mm (range, 6-21 mm). Twenty-two patients underwent interventional catheterization. Procedures included percutaneous transluminal coronary balloon angioplasty (n = 10), stent placement (n = 9), and percutaneous transluminal coronary rotational ablation (n = 3). Fourteen patients underwent coronary artery bypass graft surgery. Of the 239 patients who had coronary aneurysms with diameter > 6 mm, 13 (5.4%) presented with findings suggestive of myocardial infarction. Five patients died during the follow-up period. CONCLUSIONS: Severe stenosis or occlusion of the coronary artery may occur in some patients who develop coronary aneurysms with diameter > 6 mm; early management such as coronary interventions or surgery should be considered in such cases.

Long-term prognostic impact of dobutamine stress echocardiography in patients with Kawasaki disease and coronary artery lesions: a 15-year follow-up study.

OBJECTIVES: This study sought to determine the prognostic value of dobutamine stress echocardiography (DSE) over a 15-year follow-up for predicting cardiac events in adolescent Kawasaki disease (KD) patients with coronary artery lesions (CALs). BACKGROUND: Although DSE is an established technique for the detection of coronary artery disease, its prognostic value to predict cardiac events in adolescent KD patients with CALs is unknown. METHODS: Fifty-eight adolescent KD patients, including 36 patients with CALs documented by coronary angiography, and 22 patients with normal coronary arteries documented by echocardiography who underwent DSE were reviewed at initial testing (mean age: 13.6 years) and at 15 years' follow-up. Follow-up events were tabulated as major adverse cardiac events (MACEs) that included cardiac death, nonfatal myocardial infarction, and revascularization. RESULTS: During a mean follow-up of 14.7 years, there were 16 patients with MACEs (acute myocardial infarction: n = 1; old myocardial infarction: n = 7; coronary artery bypass grafting: n = 4; percutaneous coronary intervention: n = 4). Significant coronary artery disease (CAD) (>70% coronary stenosis) was detected in 31.0% of patients at initial testing and 42.1% at follow-up. However, there were no significant differences in wall motion score indices (WMSI) at peak DSE between initial testing and follow-up (p = 0.762). Five of 6 patients (85%) with false-positive DSE results (WMSI: ≥1.25) at initial testing, who had giant aneurysms without CAD, developed CAD with MACEs during follow-up. Cumulative event-free survival rate to 15 years was 25.0% in patients with WMSI ≥1.25 and 91.7% in patients with WMSI <1.25. Cox regression analysis showed the grade of peak WMSI at initial testing to be the only independent predictor of MACEs (relative risk: 3.28; 95% confidence interval: 1.73 to 6.20). CONCLUSIONS: DSE provided independent prognostic information up to 15 years in adolescent KD survivors.

Spontaneous coronary artery dissection: prevalence of predisposing conditions including fibromuscular dysplasia in a tertiary center cohort.

OBJECTIVES: We sought to evaluate the prevalence of fibromuscular dysplasia (FMD) and other predisposing conditions among spontaneous coronary artery dissection (SCAD) patients. BACKGROUND: Spontaneous coronary artery dissection is considered rare. However, we observed many young women with SCAD and concomitant FMD. METHODS: Spontaneous coronary artery dissection patients were identified prospectively and retrospectively at Vancouver General Hospital over the past 6 years. Coronary angiograms were meticulously reviewed by 2 senior interventional cardiologists. Identified patients were contacted for prospective evaluation at our SCAD clinic, and screening for FMD of renal, iliac, and cerebrovascular arteries was performed with computed tomography angiography or magnetic resonance angiography, if not already screened during the index angiogram. Potential predisposing and precipitating conditions for SCAD were extracted from clinical history. RESULTS: We identified 50 patients with nonatherosclerotic SCAD from April 2006 to March 2012. Average age was 51.0 years, and almost all were women (98.0%). All presented with myocardial infarction (MI), 30.0% had ST-segment elevation, and 70.0% had non-ST-segment elevation MI. Only 1 was postpartum, and 2 were involved in intense isometric exercises. Emotional stress was reported in 26.0% before the MI. Twelve percent had >1 dissected coronary artery. Most SCAD patients had FMD of ≥1 noncoronary territory (86.0%): 25 of 43 (58.1%) renal, 21 of 43 (48.8%) iliac, and 20 of 43 (46.5%) cerebrovascular (6 of 43, 14.0% had intracranial aneurysm). Five had incomplete FMD screening. CONCLUSIONS: Nonatherosclerotic SCAD predominantly affects women, and most have concomitant FMD. We suspect these patients have underlying coronary FMD that predisposed them to SCAD, but this requires proof from histology or intracoronary imaging of the affected coronary arteries.

Cardiac surgical procedures for the coronary sequelae of Kawasaki disease.

OBJECTIVES: The aim of this article is to make an evaluation on the clinical features of patients with Kawasaki disease who require a cardiac surgical procedure including coronary artery bypass grafting, coronary arterial aneurysmorrhaphy or heart transplantation. METHODS: English literature of Kawasaki disease for cardiac surgery (1990-2011) was retrieved in the Pubmed database. The clinical features of the patient setting from the representative articles were collected and analyzed. RESULTS: Patients with Kawasaki disease were very young, with some requiring a cardiac surgical procedure at a very early age. The interval between the onset and the surgical operation was 9.5±9.4 years. The prevalence of myocardial infarction and re-infarction was high. Giant aneurysm, critical stenosis with calcification and thrombus formation of the coronary arteries often warrant coronary artery bypass, heart transplantation or coronary arterial aneurysm plication. The left internal mammary artery to the left anterior descending coronary artery was the most commonly used graft in coronary artery bypass. Graft patency rate was 82.4% at 21.4±32.3 (range 0.1-252) month follow-up. The early and late mortalities of this patient setting were 0.6 and 3.0%, respectively. CONCLUSIONS: Patients with Kawasaki disease may develop coronary artery lesions prone to aneurysmal formation with calcification and thrombus and may require coronary artery bypass at a very early age. With the left internal mammary artery as the first choice of bypass graft, the long-term patency and patient survival was satisfactory.

The prevalence and clinical profile of angiographic coronary ectasia.

OBJECTIVE: To determine the incidence of Coronary Artery Ectasia (CAE) at our teaching hospital to describe the patients and angiographic characteristics. METHODS: A retrospective analysis was conducted on all coronary angiograms performed at the catheterization laboratory of Karachi Institute of Heart Diseases, a tertiary care center, between the period of August 2006 and August 2008. A one year follow up was performed to look for primary and secondary endpoints. Data were collected from catheterization films, and medical records. RESULTS: Five thousand coronary angiograms were performed during the periof of the study. A total of 140 (2.8%) angiograms showed coronary ectasia of both mixed and pure types. Pure ectasia with no coronary obstructive lesions was seen in 75 (1.5%). The left anterior descending artery (LAD) was the most commonly affected vessel (63%) followed by the right coronary artery (RCA) 25% and 10% patients had circumflex artery involvement. The primary composite endpoint showed 6 (4.2%) patients with non-ST elevation MI, 5 (3.6%) with ST elevation inferior wall myocardial infarction, 70 (50%) with unstable angina and 2 (1.4%) deaths due to pulmonary oedema. Secondary endpoints showed 50% of the patients still complaining of chest pain. CONCLUSION: Prevalence of Coronary ectasia in the population presenting to KIHD during the study period was 1.5%. Majority of patients were males, associated with dyslipidaemia, hypertension and smoking. CAE was associated with obstructive coronary artery disease in about 80% of cases. LAD was the most commonly affected vessel.