Initial intravenous immunoglobulin therapy without aspirin for acute Kawasaki disease: a retrospective cohort study with a Bayesian inference.

OBJECTIVE: To clarify the necessity of acetylsalicylic acid (ASA) administration combined with intravenous immunoglobulin (IVIG) therapy in the treatment of acute Kawasaki disease. DESIGN: Retrospective cohort study. SETTING: Multicentre. PARTICIPANTS: This study included 735 patients with Kawasaki disease aged ≤10 years and hospitalised between 4 and 10 days of illness in eight Japanese hospitals from January 2016 to December 2020. EXPOSURES: High-dose (HD) ASA was administered with initial IVIG to 333 patients in 6 hospitals (HD group). ASA was not administered routinely to 402 patients in the other two hospitals, and low-dose ASA was only administered when patients developed coronary artery lesions or pericardial effusion (non-HD group). PRIMARY AND SECONDARY OUTCOME MEASURES: The primary outcome was the presence of coronary artery lesions, defined as a coronary artery diameter >+2.5 SD of body surface area within 1 month of onset. The secondary outcome was responsiveness to the initial IVIG therapy. Adjusted risk ratios for the outcomes were calculated using modified Poisson regression models. Bayesian analysis was conducted to estimate the posterior probability of the treatment effect of HD ASA under several prior distributions. RESULTS: The incidence of coronary artery lesions was not significantly higher in the HD group than in the non-HD group (12/333 (3.6%) vs 15/402 (4.0%)). The proportion of non-responders to initial IVIG was similar between the two groups (HD group: 78/333 (23%); non-HD group: 83/402 (22%)). In the Bayesian analysis, considering a difference of ≤2% to be of no clinical importance, there was only a 9.3% chance of reduced risk of coronary artery lesions in the HD group compared with the non-HD group even with a strongly enthusiastic prior for HD treatment. CONCLUSIONS: Compared with HD ASA treatment, treatment without ASA in the acute phase of Kawasaki disease was not associated with increased complications from Kawasaki disease.

Recurrent events of acute coronary syndrome in young adult patients after Kawasaki disease.

OBJECTIVE: I encountered three adult patients with major coronary artery occlusion after Kawasaki disease in childhood, who had developed again acute coronary syndrome of adults in the peripheral branches, such as the 4th segments, the atrioventricular node artery, and the posterior descending artery, of the right coronary artery. METHODS: I reviewed their clinical course and coronary angiograms. RESULTS: Their age at onset of acute coronary syndrome ranged from 29 to 33 years. The male patient with a previous anteroseptal myocardial infarction in children had a symptomatic occlusion of the branch of the 4th posterior descending artery at 32 years of age. Acute coronary syndrome occurred in the area of 4th atrioventricular node artery in two female patients. The collateral arteries from the circumflex artery to the 4th atrioventricular node arteries were not clearly injected. It was suspected that they had developed bilateral giant aneurysms after acute Kawasaki disease. Two patients had an acute myocardial infarction due to thrombotic occlusion in a giant aneurysm of the right coronary artery or the left anterior descending artery, and one patient had an asymptomatic coronary occlusion of the right coronary artery and left anterior descending artery in children. CONCLUSION: Occlusion of peripheral coronary arteries in adulthood can occur in patients with multi-vessel disease caused by Kawasaki disease. Recurrent events of acute coronary syndrome can occur in adults, although its prevalence may be low. Careful follow-up in adults is also needed in this population.

Effect of beta-blockers and exercise restriction on the prevention of sudden cardiac death in pediatric hypertrophic cardiomyopathy.

BACKGROUND: Risk assessment tools and effective prevention strategies for sudden cardiac death (SCD) in pediatric patients with hypertrophic cardiomyopathy (HCM) have not been established. This study aimed to evaluate the efficacy of beta-blockers and exercise restriction for SCD prevention in this population. METHODS: We retrospectively reviewed the medical records of patients aged <18 years who were diagnosed with HCM at our center between January 1996 and December 2021. SCD and aborted SCD were defined as SCD equivalents. We divided patients based on whether they were prescribed beta-blockers or exercise restriction and compared the outcomes among the groups. The primary outcome was the overall survival (OS), and the secondary outcome was the cumulative SCD equivalent rate. Outcomes were analyzed using Kaplan-Meier curves and Cox proportional hazard analysis. We also compared patients according to the occurrence of SCD equivalents to identify SCD risk predictors. RESULTS: Among the 43 included patients [mean age, 7.7 (1.6-12.1) years; 23 male individuals], SCD equivalents occurred in 13 patients over 11.2 (4.5-15.6) years of follow-up, among whom 12 were resuscitated and 1 died. The OS rate was significantly higher in the beta-blocker and exercise restriction groups than in the non-beta-blocker and non-exercise restriction groups (81.3 % vs. 19.1 %, p < 0.01 and 57.4 % vs. 12.7 %, p < 0.01, respectively). Among the 13 patients with SCD equivalents, 5 had 9 recurrent SCD equivalents. A significant difference was observed between the SCD equivalent and non-SCD equivalent groups in the history of suspected arrhythmogenic syncope (p < 0.01) in the univariable but not in the multivariable analysis. CONCLUSIONS: Beta-blockers and exercise restriction may decrease the risk of SCD in pediatric patients with HCM and should be considered for SCD prevention in this population, particularly because predicting SCD in these patients remains challenging.

Clinical Course and Outcomes in Patients with Left Ventricular Dysfunction Due to Myocardial Infarction After Kawasaki Disease.

Long-term outcomes of patients with left ventricular dysfunction after Kawasaki disease (KD) remain unknown. The clinical course of 37 patients (29 males 8 females) with left ventricular ejection function (LVEF) less than 45% after KD since 1978 was retrospectively investigated. Cardiac events in this study were defined as admissions because of heart failure, fatal ventricular arrhythmias, heart transplantation, and death. Their ages when evaluated ranged from 1 to 70 years (median 35 years). The onset of KD ranged from 2 months to 20 years (median 21 months). All patients had bilateral coronary artery lesions, and multi-vessel occlusion occurred in 31 patients (84%). Previous myocardial infarction (MI) was found in 28 patients (76%). The interval from the onset of acute KD to the initial MI ranged from 15 days to 25 years (median 3 months). Reperfusion therapy was successful in 6 patients (30%), and coronary artery bypass grafting was performed in 23 patients (62%). Non-sustained ventricular tachycardia and fatal ventricular arrhythmias were detected in 11 patients and 22 patients, respectively. There were 15 deaths (41%). The 20-year and 50-year survival rates after KD were 84% (95% CI 67-92) and 54% (34-73), respectively (n = 37). The 30-year cardiac event-free rate after the detection of low LVEF was 42% (95% CI 27-59). The cutoff point of the left ventricular end-diastolic dimension for cardiac events was 65 mm. Patients with low LVEF had fatal ventricular arrhythmias and a worsening of their ischemic cardiomyopathy after 30 years of age and their outcomes were poor.

Characteristics and long-term outcome for congenital left main coronary artery atresia.

The prevalence of congenital left main coronary artery atresia is very low. We report the characteristics and long-term outcomes of four children with left main coronary artery atresia. Three patients had heart murmurs due to mitral regurgitation at less than 1 year old. Their myocardial ischaemia worsened on exercise with aging. In the fourth patient, hypertrophic cardiomyopathy and Noonan syndrome were suspected at 1 year old. The development of communicating arteries between the conus branch and the left anterior descending artery was detected at 7 years old. The left main coronary artery atresia was confirmed by a selective coronary angiogram at 15 years old. Congenital left main coronary artery atresia could not be diagnosed by two-dimensional echocardiography; however, the left coronary arteries were small. Two patients underwent coronary artery bypass grafting of the left anterior descending artery using the left internal thoracic artery at 3 years and 6 years old, respectively. Two patients had an angioplasty with a cut back at the orifice of the left coronary artery at 2 years old and 17 years old, respectively. Two patients had no cardiac events without medication for more than 30 years after the operation. We must differentiate the diagnosis of left main coronary artery atresia in the small left coronary arteries with mitral regurgitation during the first year. Coronary artery revascularisation and mitral annuloplasty are needed. The long-term outcome of both coronary artery bypass grafting and angioplasty were good. The degree of mitral regurgitation after surgery may affect the prognosis.

Long-term results of large common iliac artery aneurysms caused by Kawasaki disease in four patients.

Among Kawasaki disease patients with systemic artery aneurysms, the brachial and internal iliac arteries are the most commonly affected, and occlusions of both arteries are often found. However, the long-term fate of large common iliac artery aneurysms remains unknown, because their prevalence is very low. The long-term outcomes of common iliac artery aneurysms caused by Kawasaki disease in four patients (three females, one male) were investigated retrospectively based on their medical records and angiograms. Their ages ranged from 30 to 36 years-old. The onset age of Kawasaki disease ranged from 4 to 8 months, and the interval from the onset of Kawasaki disease to the latest angiogram ranged from 17 to 21 years. All patients had bilateral large coronary aneurysms and common iliac artery aneurysms with maximal diameters greater than 10 mm. Although all patients had multi-vessel coronary artery stenotic lesions and systemic artery aneurysms, they were asymptomatic. The three female patients underwent coronary artery bypass grafting, and the male patient underwent replacement of artificial vessels for large bilateral common iliac artery aneurysms at 3 years old of age. Over the long-term, common iliac artery aneurysms greater than 10 mm persisted as calcified aneurysms. However, they had no symptoms due to their common iliac artery aneurysms, and their ankle brachial pressure index was preserved, even if the stenosis of the common iliac artery developed as a late outcome, because the collateral arteries were well developed. The progression of stenosis of the common iliac artery after Kawasaki disease was slower.

Thirty-year outcome in children with hypertrophic cardiomyopathy based on the type.

BACKGROUND: We reviewed the long-term outcome of children with hypertrophic cardiomyopathy (HCM) based on the type. METHODS: We reviewed the medical records of 100 patients (male 54 female 46) with HCM at our hospital between 1977 and 2015. The survival and cardiac event-free survival rates were calculated by the Kaplan-Meier method. RESULTS: The age at the time of the diagnosis ranged from 0 to 15 years with a median of 8 years. The number of patients with Noonan syndrome and hypertrophic obstructive cardiomyopathy (HOCM), idiopathic HCM (i-HCM), and secondary HCM (s-HCM) was 13, 13, 65, and 9 respectively. A dilated phase of HCM occurred in 24 patients. Nineteen (79 %) of the 24 patients died of heart failure, and two underwent a heart transplantation. Eight (33 %) of the 24 patients had s-HCM. The median age when a dilated phase occurred was 15 years old, and the median interval from the initial diagnosis to the dilated phase was 8 years. The median time from the diagnosis of a dilated phase to death was 1.6 years. Sudden death and implantable cardioverter defibrillator implantations occurred in 6 and 11 patients at around 15 years old, respectively. The 20-year survival rates were as follows: Noonan syndrome 84 %; HOCM 82 %; i-HCM 71 %; and s-HCM 17 %. Overall, the survival rates at 10, 20, and 30 years were 83 % (95 % confidence interval 73-89), 69 % (58-78), and 63 % (50-74), respectively. The overall cardiac event-free survival rates at 10, 20, and 30 years were 57 % (47-67), 39 % (31-50), and 32 % (21-44), respectively. CONCLUSION: The long-term outcome in children with HCM was poor, and the outcome of s-HCM was very poor. The occurrence of a dilated phase worsened the outcome in HCM patients. Sudden death and d-HCM often occurred at around 15 years old.

Coronary artery aneurysms of unknown origin in a 14-year-old girl.

The case of a 14-year-old girl with acute myocardial infarction due to coronary artery aneurysms (CAAs) of unknown origin, which resembled coronary artery lesions caused by Kawasaki disease, is reported. She was transferred to our hospital due to chest pain with ST-T elevation. She had no history of Kawasaki disease. On the first admission, she was misdiagnosed with acute myocarditis. Then, 54 days later, she experienced chest pain with exertional dyspnea. Her electrocardiogram showed negative T waves in the chest leads. A CAA of the left coronary artery was suspected on two-dimensional echocardiography. Coronary angiograms showed 90% stenosis and multiple CAAs of the left anterior descending artery and the bifurcation of the left coronary artery. Both the right coronary artery and left circumflex artery were occluded. A left ventriculogram showed dyskinesis and an aneurysm at the apex. She underwent triple-vessel coronary artery bypass grafting, and her symptoms improved. In addition, an intracranial aneurysm was also found on cerebral angiography. There were no specific laboratory findings other than SS-A antibodies. It was suspected that the weakness of the vessels was related to the disease. It may have been a different disease that was never previously detected, but her CAAs were Kawasaki-like CAAs.

Long-term results of percutaneous transluminal coronary balloon angioplasty in Kawasaki disease.

We report the long-term results of percutaneous transluminal coronary balloon angioplasty for early appearing localised stenosis of the left anterior descending artery caused by Kawasaki disease in two 2-year-olds. One patient had 50% restenosis and newly appearing aneurysm at 21-year-old, and the other had no restenosis at 16-year-old. They had no symptoms or cardiac events for 20 years. It is useful in the early localised stenosis without aneurysm.

Long-term results of percutaneous transluminal coronary rotational atherectomy for localised stenosis caused by Kawasaki disease.

Thirteen boys and one girl, 5-30 years (median 13 years), underwent percutaneous transluminal coronary rotational atherectomy. The interval from the onset of Kawasaki disease to PTCRA ranged from 5 to 29 years (median 12 years). The follow-up period was 1-22 years (median 13 years). The target vessels were the right coronary artery (7), left anterior descending artery (3), left circumflex (2), and left main trunk (2). The maximum burr size used was 1.75 mm in four, 2.00 mm in four, and 2.15 mm in six. The immediate results of rotational atherectomy were successful in all patients, and the mean stenosis degree improved from 86 ± 15% (mean ± standard deviation) to 37 ± 14% (p < 0.001). Cardiac events in the late period were found in four patients (29%). Acute myocardial infarction occurred in two, and syncope and ventricular fibrillation in one each. The cardiac event-free rate at 10 and 20 years was 79% (95% confidence interval 50-92) and 39% (6-87), respectively, (n = 14). The overall 20-year patency rate was 54% (95% CI 28-78). That in patients more than 10 years old was 77% (95% CI 42-94, n = 10). PTCRA alone is suitable for severe localised stenosis with calcification caused by KD in young adults except for small children. Re-stenosis within the first year after PTCRA often develops because of reactive intimal thickening after the procedure. If a target vessel is a patent 1 year after the procedure, long-term patency may be expected in patients more than 10 years old.

Characteristics of coronary flow velocity reserve on transthoracic Doppler echocardiography in patients with a history of Kawasaki disease.

Whether the acute inflammation caused by Kawasaki disease will have an effect on the endothelial function of coronary arteries in the future is unknown. METHODS: The coronary flow velocity reserve of the left anterior descending artery was examined by transthoracic Doppler echocardiography in 189 patients (male 125, female 64) with a history of Kawasaki disease and 10 volunteers (control). Their ages ranged from 6 to 40 years (median 22 years). The patients were divided into nine groups based on the left anterior descending artery lesions. The coronary flow velocity reserve was measured by intravenous administration of adenosine triphosphate (0.15 mg/kg/minute) while fasting. The coronary flow velocity reserve was calculated as the ratio of hyperaemic to basal mean diastolic flow velocities. The respective groups were as follows: control, no coronary artery lesions (n = 39), no coronary artery lesions in the right coronary artery (n = 29), regression (n = 11), aneurysm at the bifurcation of the left coronary artery (n = 26), aneurysm of the left anterior descending artery (n = 15), localised stenosis <75% (n = 12), localised stenosis ≥75% (n = 17), segmental stenosis (n = 5) and coronary artery bypass grafting (n = 36). One-factor ANOVA followed by Tukey's test was used to compare the coronary flow velocity reserve among the groups. RESULTS: The coronary flow velocity reserve was significantly lower in the localised stenosis ≥75%, segmental stenosis and coronary artery grafting groups than in the other groups (p < 0.05). CONCLUSIONS: The endothelial function in the epicoronary artery was preserved in patients with a history of Kawasaki disease and dilated coronary artery lesions.

Changes in Coronary Aneurysm Diameters After Acute Kawasaki Disease from Infancy to Adolescence.

We clarified the changes in coronary artery diameters based on the degree of coronary artery involvement immediately after acute Kawasaki disease (KD). Two hundred sixteen coronary arteries in 85 patients after KD examined by two-dimensional echocardiography were reviewed from 1995. The maximal internal diameters were measured at 2 months, 1 year, 3 years, 10 years and 15 years after KD. The coronary arteries were divided into five groups based on the absolute diameter at 2 months, as well as six groups based on the Z score at 2 months. The maximum diameters were compared at 2 months with those during follow-up in each group. The numbers of right coronary, left anterior descending, left coronary, and left circumflex arteries were 84, 73, 55 and 4, respectively. There was a significant relationship between the maximum internal diameter at 2 months and subsequent changes in the maximum diameters after KD in the late period in both groups (p < 0.0001). The maximum diameters of coronary artery aneurysms (CAAs) ≥ 8.0 mm at 2 months did not change significantly after 1 year, however, the maximum diameter of CAAs < 8.0 mm was significantly smaller in the late period (p < 0.05). Coronary arteries without dilatation at 2 months after KD grew normally. CAAs with a maximum diameter ≥ 6.0 mm and Z score ≥ 7.5 at 2 months after KD persisted in adolescents, whereas coronary arteries with diameters < 6.0 mm and Z score < 7.5 could be within normal ranges in the late period.

Time Course of Coronary Artery Aneurysms in Kawasaki Disease.

OBJECTIVES: To determine the timeframe in which coronary artery aneurysms (CAAs) caused by Kawasaki disease reach their maximum diameter, the timeframe in which they regress to normal size, and the cutoff point of the diameter for CAA regression. STUDY DESIGN: We reviewed 195 CAAs of the right coronary artery, left anterior descending artery, and left coronary artery measured by 2-dimensional echocardiography ≥5 times for 1 year after Kawasaki disease in 84 patients using medical records from 1995. The maximum diameters of CAAs were investigated retrospectively. The time to CAA regression using both absolute diameter and Z score were investigated. The cutoff points of the diameter of CAA regression in the 2 classifications were identified using receiver operator characteristic curve analysis. One year after Kawasaki disease, a CAA of <3.0 mm in absolute diameter and a Z score of <2.5 were defined as CAA regression. RESULTS: The time when CAAs reached their maximum diameter ranged from 11 days to 87 days, with a median of 35 days (n = 195). The time to CAA regression ranged from 41 to 386 days, with a median of 136 days in the absolute diameter classification (n = 92); 78% of CAA regression regressed by 200 days. The cutoff point for CAA regression at one year was 5.7 mm for the absolute diameter (area under the curve, 0.887; P < .0001; n = 190) and 9.5 for the Z score (area under the curve, 0.815; P < .0001; n = 195). CONCLUSIONS: CAAs with a smaller diameter regressed earlier, and most CAAs of <6 mm regressed by 6 months after Kawasaki disease.

Cardiac events in Patients in their forties with Kawasaki disease and regression of coronary artery aneurysms.

Over a 50-year period from the first description of Kawasaki disease, we encountered three male patients with a history of Kawasaki disease, who had their first cardiac events in their forties. They were considered to have almost normal coronary arteries in the coronary angiograms when they were children and adolescents. They had no follow-up examinations after 20 years old. The 1st patient had an acute myocardial infarction, and the 2nd was a new appearance of coronary aneurysm and stenotic lesions with coronary artery calcification. The 3rd patient had unexpected sudden death. The interval from the onset of Kawasaki disease to the cardiac events ranged from 37 to 38 years. In the former two patients, coronary artery lesions could not be evaluated immediately after Kawasaki disease. Although the 3rd patient had bilateral medium-sized coronary artery aneurysms, his coronary aneurysms regressed 1 year after acute Kawasaki disease. The intimal thickening at a previous coronary aneurysm at the age of 19 was mild. The patients with regressed coronary aneurysms were asymptomatic for about 40 years after Kawasaki disease, prior to their cardiac events. Coronary artery calcification of the proximal portion of the major coronary arteries was a predictable marker in such patients. To prevent serious cardiac events in middle-aged adult patients, reevaluation of coronary artery lesions and restarting of anti-thrombotic therapy are needed. We must be aware that there are some differences in the clinical course and time of cardiac events between patients with giant aneurysms and those with medium aneurysms.

Etiology of atrial fibrillation in patients with complex congenital heart disease - for a better treatment strategy.

BACKGROUND: The demographics of patients with congenital heart disease (CHD) and atrial fibrillation (AF) differ significantly from the general population. The etiology and treatment strategy for AF in CHD patients have been investigated but are to date inconclusive. METHODS: To determine the etiology of AF in CHD and to seek a better treatment strategy, we retrospectively evaluated the atrial overload in 42 complex CHD cases with normal atrial arrangements and AF (age 25; range, 9-66 years) and the impact of a reduction in the atrial overload on the atrial rhythm. RESULTS: Cardiac defect diagnoses varied, with 17% of the patients having a persistent left superior vena cava (PLSVC). In regard to the volume overload, the frequencies of an overload in the right atrium (RA), left atrium (LA), or both, were 50 %, 23%, and 10%, respectively (p = 0.015). Other sustained supraventricular tachycardias were observed in 29 patients (69%) before and after the onset of AF. Among these 29 patients, 26 had intra-atrial reentrant tachycardia. Fifteen patients (36%), 10 of whom had chronic AF, died during the follow-up including 3 with arrhythmias and 10 because of heart failure. Fourteen (33%) patients had no AF at the last follow-up due to medical interventions, 8 of which underwent solely an RA-sided catheter ablation and/or surgical RA overload reduction. CONCLUSIONS: AF in complex CHD with a normal atrial arrangement correlates with a higher RA-sided overload than an LA-sided and exhibits a high incidence of PLSVCs, high comorbidity of intra-atrial reentrant tachycardias, and high mortality rate. In a substantial number of patients, RA-sided interventions were effective in controlling AF. To effectively manage AF in complex CHD it is essential to understand each individual's hemodynamics and consider hemodynamic interventions.

Insights into stent implantation for coronary artery lesions caused by Kawasaki disease.

Fifty years have passed since the first report of Kawasaki disease in 1967, and the prevalence of acute coronary syndrome in Kawasaki disease patients with coronary artery lesions exceeding 40 years old has increased. Primary coronary stent implantation is currently an acceptable method in ischaemic coronary heart disease in adults. However, it is unknown whether the stent implantation is effective or not in this population. As the clue to answer this question, I reviewed the references on Kawasaki disease patients who underwent the stent implantations between 1997 and 2019. Thirty-three patients underwent stent implantations for 34 coronary arteries. Adverse effects in the late period were found in 19 (68%) of 28 vessels with follow-up angiograms. There were complete occlusion 9, restenosis 8, and migration 2. A new aneurysm formation was found in 7 (37%) among the 19 vessels, and 6 (86%) of the 7 vessels were drug-eluting stent and 5 were found after the procedure for chronic total occlusion. The adverse effects free-rate at 1 year and 3 years were 57 and 25%, respectively. At present, the usefulness of stent implantation in the long-term results was scarce. Even if primary percutaneous coronary intervention without a stent implantation is performed for acute coronary syndrome, it can be expected to maintain the patency of the culprit lesion for several years. It is better to avoid a stent implantation as long as possible in this population. Knowing the long-term efficacy and complications of stent implantations is important for deciding the procedure.

Effect of Stiffened and Dilated Ascending Aorta on Aerobic Exercise Capacity in Repaired Patients With Complex Congenital Heart Disease.

Several studies have reported aortic dilation and increased stiffness of the ascending aorta in patients after repair of congenital heart disease (CHD), which may be a predominant cardiovascular risk. However, the clinical significance has not been described in detail. In this retrospective study, 175 repaired patients with complex CHD achieving biventricular circulation and age-matched 39 control subjects were reviewed (median age: 14.9 and 15.7 years, respectively). We measured the diameters of the ascending aorta and descending aorta from catheterization angiograms to yield Z-scores and stiffness indexes (ß) using diameter fluctuations corresponding to pulsatile pressures. Clinical profile, peak oxygen uptake during the cardiopulmonary exercise test, and incidence of unscheduled hospitalization during follow-up was also reviewed. Compared with controls, patients with complex CHD, except for those with aortic coarctation, exhibited significant dilation and increased stiffness of the aortic root and ascending aorta, but not of the descending aorta. In this CHD population (n = 147, including 112 conotruncal anomalies), exercise capacities correlated independently with the diameter Z-score and stiffness index of the ascending aorta along with the history of repetitive thoracotomies, reduced forced vital capacity, and right ventricular hypertension. During a follow-up period (median 15.6 years), either dilation (Z-score >3.5) or increased stiffness (ß >6.0) of the ascending aorta stratified morbidity, but no synergistic impact was detected. In conclusion, in repaired patients with complex CHD, a stiffened and dilated ascending aorta was frequently found, exerting significant adverse impacts on diminished exercise capacity and morbidity.