Case Report: Interventional therapy for coronary artery occlusion in a 6-year-old child with Kawasaki disease.

A 6-year-old girl was diagnosed with Kawasaki disease and bilateral giant coronary artery aneurysms at four months old and was subsequently referred to our hospital due to chest pain and T wave changes on electrocardiography. After admission, stress myocardial perfusion imaging showed reversible ischemia in multiple areas of the left ventricle. Coronary angiography revealed complete proximal segment occlusion of the left circumflex artery (LCX). The occluded LCX was recanalized by a Gaia 3rd micro-wire successfully passing through the occluded section to the distal end of the LCX, followed by sequential balloon dilation and drug-coated balloon angioplasty. Coronary angiography immediately after post-dilation and one-year follow-up angiography showed that the structure and blood flow of LCX was good. Although percutaneous coronary intervention (PCI) in pediatric patients with Kawasaki disease is limited in practice, PCI remains one of the treatment options for selected patients.

Tenascin-C predicts IVIG non-responsiveness and coronary artery lesions in kawasaki disease in a Chinese cohort.

Objectives: To assess the predictive value of tenascin-C (TN-C) for intravenous immunoglobulin (IVIG) non-responsiveness and coronary artery lesions (CALs) development at the acute stage of Kawasaki disease, and to build novel scoring systems for identifying IVIG non-responsiveness and CALs. Methods: A total of 261 patients in acute-stage Kawasaki disease were included. Serum samples before IVIG initiation were collected and TN-C expression levels were measured using an enzyme-linked immunosorbent assay. In addition to TN-C, another fifteen clinical and laboratory parameters collected before treatment were compared between IVIG responsive and non-responsive groups, and between groups with and without CALs. Multiple logistic regression analyses were performed to construct new scoring systems for the prediction of IVIG non-responsiveness and CALs development. Results: IVIG non-responsive group (n = 51) had significantly higher TN-C level compared to IVIG responsive group (n = 210) (15.44 vs. 12.38 IU/L, P < 0.001). A novel scoring system composed of TN-C, total bilirubin, serum sodium and albumin was established to predict IVIG non-responsiveness. Patients with a total score ≥ 2 points were classified as high-risk cases. With the sensitivity of 78.4% and specificity of 73.8%, the efficiency of our scoring system for predicting IVIG non-responsiveness was comparable to the Kobayashi system. Consistently, the group developing CALs at the acute stage (n = 42) had significantly higher TN-C level compared to the group without CALs (n = 219) (19.76 vs. 12.10 IU/L, P < 0.001). A new scoring system showed that patients with elevated TN-C, platelet count ≥ 450 × 109/L, and delayed initial infusion of IVIG had a higher risk of developing CALs. Individuals with a total score ≥ 3 points were classified as high-risk cases. The sensitivity and specificity of the novel simple system for predicting CALs development were 83.3% and 74.0%, respectively, yielding a better efficiency than the Harada score. Conclusion: Elevated TN-C appeared to be an independent risk factor for both IVIG non-responsiveness and CALs in Chinese children with KD. Our scoring systems containing TN-C is simple and efficient in the early identification of high-risk KD cases that could benefit from more individualized medications.

Mid-aortic syndrome: a rare cause of heart failure in infants.

This case reports describe a rare disease, mid-aortic syndrome (MAS), that can cause severe heart failure and hypertension in infancy. The typical images, key points of diagnosis, and therapy methods of the disease have also been presented. We report two critical thoracoabdominal aortic coarctation cases in infants aged 2 and 11 months with severe heart failure. The patients were initially misdiagnosed as dilated myocardiopathy, with the correct diagnosis confirmed through imaging. Both patients underwent balloon angioplasty; one patient also had bare-metal stents implanted. The patient treated with balloon angioplasty alone died after the procedure, whereas the other patient recovered well. In conclusion, careful physical examinations, especially upper and lower extremity blood pressure differences and palpation of upper and lower limb pulses, are critical in unexplained infant heart failure cases. Stent implantation may be a safer and more effective treatment than simple balloon angioplasty in infants with MAS.

Case Report: Recurrent Left Posterior Fascicular Ventricular Tachycardia in a Newborn.

Left posterior fascicular ventricular tachycardia (LPFVT) is extremely rare in neonates. We described a 17-day-old girl with LPFVT who was initially misdiagnosed as supraventricular tachycardia (SVT). Eventually, she was successfully treated by amiodarone infusion followed by oral amiodarone with propranolol for 9 months, and LPFVT spontaneously resolved after a 1-year follow-up. This case report illustrated the basic principles and caveats in differential diagnosis of LPFVT in the neonatal age group. With proper diagnosis and therapy, neonatal LPFVT might regress in the first year of life.

Randomized trial of different initial intravenous immunoglobulin regimens in Kawasaki disease.

BACKGROUND: We aimed to assess the efficacy of different initial intravenous immunoglobulin (IVIG) regimens in Kawasaki disease (KD) patients to find more cost-effective therapy options. METHODS: A multicenter, open-label, blind-endpoint randomized controlled trial was conducted from January 2014 to December 2015. Patients with KD, within 10 days of illness, were randomly assigned to receive different IVIG regimens (Group A, 2 g/kg once; Group B, 1 g/kg for 2 consecutive days; Group C, 1 g/kg once) and aspirin 30mg/kg/d. Primary outcomes included hours to defervescence and development of coronary artery lesions during the study period. Major secondary outcomes included total fever days, total dose of IVIG, changes of laboratory data, length of stay, and hospitalization expenses. (ClinicalTrials.gov: NCT02439996). RESULTS: A total of 404 patients underwent randomization. No difference was found in the outcomes of defervescence among three groups at 6, 12, 24, and 36 hours after completion of initial IVIG infusion. There were no differences in the incidence of coronary artery lesions during the study period (at week 2, month 1, month 3, and month 6 of illness), changes of laboratory data, total fever days, and length of stay. Group C patients had the lowest total dose of IVIG (mean: 1.2 vs 2.2 vs 2.1 g/kg; P < 0.001) and hospitalization expenses (mean: 8443.8 vs 10798.4 vs 11011.4 Chinese Yuan; P < 0.001) than other two groups. CONCLUSIONS: A single dose of 1g/kg IVIG is a low-cost treatment with the same efficacy as 2 g/kg IVIG and can be an option for the initial therapy of KD patients.

Neonatal Kawasaki disease with multiple arterial aneurysms: a case report.

BACKGROUND: Kawasaki disease (KD) is a medium vessel vasculitis that typically occurs in children aged between 6 months and 5 years. It is extraordinarily rare in the neonatal period. KD-related systemic artery aneurysms (SAAs) have never been reported in neonates. CASE PRESENTATION: A male infant was transferred to our institution for persistent high-grade fever lasting 16 days. Symptoms started at day 14 of life, and he was admitted to a children's hospital on the second day of fever. Physical examination at the time found no signs suggestive of KD. The only laboratory parameters which were of significance were values suggestive of systemic inflammation. However, his fever persisted and inflammatory markers continued to rise despite 2 weeks of antibiotic therapy. KD as a noninfectious cause of fever was considered when he came to our institution, and echocardiographic findings of left and right medium coronary artery aneurysms (CAAs) confirmed our suspicions. Full-body magnetic resonance angiography also revealed bilateral axillary artery aneurysms. Administration of intravenous gamma globulin resulted in rapid improvement. His fever resolved on the next day and CAAs and SAAs regressed to normal at 6 months and 3 months after diagnosis, respectively. CONCLUSION: This unique case of incomplete KD highlights the importance of considering KD in neonates with unexplained prolonged fever and reinforces the need to remain vigilant for SAAs in KD.

Systemic Artery Aneurysms and Kawasaki Disease.

BACKGROUND: Coronary artery aneurysms (CAAs) are a well-known complication of Kawasaki disease (KD), but there are no data on incidence or outcomes of systemic artery aneurysms (SAAs) in the current era. METHODS: From April 1, 2016, to March 31, 2019, we screened for SAAs in 162 patients with KD at risk for SAAs with magnetic resonance angiography or peripheral angiography and analyzed incidence and early outcomes of SAAs. RESULTS: Twenty-three patients had SAAs, demonstrating an incidence of 14.2% (23 of 162) in patients who were screened at 1 month after onset. The proportion of patients with SAAs was estimated to be 2% (23 of 1148) of all patients with KD. The median age at onset of KD with SAAs was 5 months. All patients with SAAs had CAAs, with z scores >8. Of patients with giant CAAs, 38.6% (17 of 44) had SAAs. A total of 129 SAAs occurred in 17 different named arteries. The most common sites for SAAs were the axillary (18.6%), common iliac (12.4%), and brachial (11.6%) arteries. During a median follow-up time of 6 months, 92.9% (79 of 85) of SAAs had some degree of regression, with 80% (68 of 85) of SAAs returning to normal. The overall regression rate was higher for medium to large SAAs than for medium to giant CAAs. CONCLUSIONS: Although the incidence of SAAs may not be as dramatically reduced as we expected compared with previous data, SAAs have a high regression rate during short-term follow-up.

Pulse Oximetry and Auscultation for Congenital Heart Disease Detection.

OBJECTIVES: Pulse oximetry (POX) has been confirmed as a specific screening modality for critical congenital heart disease (CCHD), with moderate sensitivity. However, POX is not able to detect most serious and critical cardiac lesions (major congenital heart disease [CHD]) without hypoxemia. In this study, we investigated the accuracy and feasibility of the addition of cardiac auscultation to POX as a screening method for asymptomatic major CHD. METHODS: A multicenter prospective observational screening study was conducted at 15 hospitals in Shanghai between July 1, 2012, and December 31, 2014. Newborns with either an abnormal POX or cardiac auscultation were defined as screen positive. All screen-positive newborns underwent further echocardiography. False-negative results were identified by clinical follow-up, parents' feedback, and telephone review. We assessed the accuracy of POX plus cardiac auscultation for the detection of major CHD. RESULTS: CHD screening was completed in all 15 hospitals, with a screening rate of 94.0% to 99.8%. In total, 167 190 consecutive asymptomatic newborn infants were screened, of which 203 had major CHD (44 critical and 159 serious). The sensitivity of POX plus cardiac auscultation was 95.5% (95% confidence interval 84.9%-98.7%) for CCHD and 92.1% (95% confidence interval 87.7%-95.1%) for major CHD. The false-positive rate was 1.2% for detecting CCHD and 1.1% for detecting major CHD. CONCLUSIONS: In our current study, we show that using POX plus cardiac auscultation significantly improved the detection rate of major CHD in the early neonatal stage, with high sensitivity and a reasonable false-positive rate. It provides strong evidence and a reliable method for neonatal CHD screening.

Surgical Outcomes of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Children: An Echocardiography Follow-up.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially life-threatening congenital heart defect. A retrospective analysis was carried out to elucidate the surgical outcomes of ALCAPA in infants and children using follow-up echocardiography. METHODS: From September 2008 to March 2017, 26 children diagnosed with ALCAPA underwent left coronary re-implantation. All surviving patients received echocardiography during follow-up. RESULTS: The mortality rate after the operation was 11.5%. Before repair, twenty patients (76.9%) presented with left ventricular (LV) dysfunction. The mean Z-score of the preoperative LV end-diastolic diameter was 4.42 ± 2.09. Mitral regurgitation (MR) was present in all patients. Two patients (7.7%), both with mitral valve prolapse, underwent mitral valve repair at the time of ALCAPA repair. Two children required postoperative extracorporeal membrane oxygenation. LV function normalized at a median time of 5.3 months (range: 0.5-36.0 months). The Z-score of the LV end-diastolic diameter decreased simultaneously. The degree of MR gradually decreased in all surviving patients. All patients had patency of the proximal left coronary artery confirmed by echocardiography at the most recent follow-up. Six patients (26.1%) showed supravalvar pulmonary stenosis and seven patients (30.4%) showed right pulmonary stenosis during follow-up. CONCLUSIONS: Coronary re-implantation was effective for rebuilding a dual coronary system in patients with ALCAPA and resulted in progressive improved LV function and reduced functional MR. Echocardiography was valuable for evaluating the outcomes. LV function, the degree of MR, and possible complications could be detected with follow-up echocardiography.

Successful ablation of incessant idiopathic right ventricular tachycardia arising from unusual sites in children.

OBJECTIVE: Most idiopathic right ventricular tachycardias originate from the outflow tract. We present a case series of idiopathic incessant ventricular tachycardia arising from unusual sites of the right ventricle in children, which were well resolved by catheter ablation. METHODS: A retrospective review was performed of all three patients who underwent ablation of idiopathic ventricular tachycardia below the level of the right ventricular outflow tract using three-dimensional mapping in our institute. Result All three patients presented with tachycardia-induced cardiomyopathy due to incessant ventricular tachycardia on first admission. The sites of successful ablation were at the proximal right bundle branch, distal right bundle branch, and apex of the right ventricle, respectively. No complications occurred, and there has been no recurrence of ventricular tachycardia after the final ablation at an average follow-up period of 9 months. All three patients have achieved normalisation of left ventricular size and systolic function. CONCLUSION: Incessant idiopathic ventricular tachycardia originating from unusual sites of the right ventricle in children, resulting in significant symptoms and impaired ventricular function, can be successfully treated with catheter ablation.

Anomalous left coronary artery arising from the right pulmonary artery in association with coarctation of the aorta.

In this study, we describe a very rare case of anomalous left coronary artery arising from the right pulmonary artery in association with coarctation of the aorta. A 3-month-old boy presented with refractory congestive heart failure since 20 days after birth. The initial echocardiography suggested the diagnosis of left coronary artery-to-right pulmonary artery fistula associated with coarctation; however, selective coronary angiography demonstrated the rare anomaly of the left coronary artery arising from the right pulmonary artery. Subsequently, he underwent successful transcatheter balloon angioplasty for aortic coarctation and surgical repair of left coronary artery re-implantation.

[Diagnosis of noncompaction of the ventricular myocardium by echocardiography].

OBJECTIVE: To evaluate the value of echocardiography in the diagnosis of noncompaction of the ventricular myocardium (NCVM) and to elucidate the echocardiographic characteristics of NCVM. METHODS: This study included 53 patients (28 boys and 25 girls), with an age for initial diagnosis of 15 days to 18 years, who were diagnosed with NCVM by echocardiography between May 2006 and May 2015. Transthoracic two-dimensional echocardiography and color Doppler were performed for qualitative diagnosis, and the end-diastolic non-compacted layer/compacted layer (N/C) ratio measured in the parasternal ventricular short-axis sectional view was selected as the criterion for quantitative diagnosis. RESULTS: The excessively prominent ventricular trabeculae and deep inter-trabecular recesses were all seen in 53 cases, and the blood flow in the cardiac chambers was connected to the inter-trabecular recesses. The areas involved in NCVM were mainly the apex (100%) and the middle segment of the left ventricular lateral wall (98%), followed by the middle segment of the left ventricular posterior wall (49%) and the middle segment of the left ventricular inferior wall (42%). In 53 children with NCVM, the N/C ratio was 4.3±1.9 (2.1-10.0). Cardiac insufficiency was found in 83% (44/53) of the children with NCVM, and the left ventricular ejection fraction for these children was (43±9)%. CONCLUSIONS: Echocardiography can be used in the qualitative and quantitative diagnosis of NCVM and in the evaluation of cardiac function. The apex and the middle segment of the left ventricular lateral wall are often involved in NCVM, accompanied by decrease in the left ventricular ejection fraction.

[Evaluation of treatment and management of Kawasaki coronary artery disease based on clinical severity classification].

OBJECTIVE: To evaluate the efficacy and safety of evaluation,treatment and follow-up of Kawasaki coronary artery disease based on the clinical severity classification. METHOD: This study evaluated 52 patients admitted to the Children's Hospital of Fudan University between July 2005 and December 2013 who were diagnosed with Kawasaki Disease with coronary artery disease.Inclusion criteria were a disease course of more than two months, initial echocardiography showing severity of grade IV and above, and confirmation of disease severity by angiography. Of those studies, 44 were male and eight were female, aged 6 to 142 (average 41) months. Treatment was planned according to protocols in "Suggestions for Management of Kawasaki Coronary Artery Disease" with follow-up. Those patients with grade IV and above confirmed by angiogram were given oral low-dose asprin and warfarin, and those with grade Vb were given coronary artery bypass grafting (CABG) after comprehensive evaluation. Analysis was carried out for diagnosis, treatment, complications, and results of follow-up. RESULT: (1) Satisfied images were shown by the angiography of all 52 cases. Forty five patients (86%) had giant aneurysm or multiple aneurysms, with thrombosis found in 10 of 45 patients (22%). Coronary artery lesions occurred in 138 coronary branches, and more common in left anterior descending branch (47 branches, with incidence 34%) and right coronary artery (48 branches, with incidence 35%). There were no complications during or after angiography. (2) After angiography, 49 patients remained at grade IV or above, and three improved to grade III. Ultimately, clinical severities of coronary artery disease included three patients at grade III, 31 patients at grade IV, nine patients at grade Va, and nine patients at grade Vb. (3) Thirty-eight patients were properly using aspirin and warfarin, and two patients with severely elevated international normalized ratio (INR) levels presented with knee joint and gastric hemorrhage, both of which were treated successfully.Patients with INR levels between 1.5 and 2.5 did not show signs of hemorrhage. (4) In follow-up visits between 6 months and 8 years, one patient had representation of thrombosis on angiography, but did not lead to coronary stenosis; four patients were improved from grade IV to either grade III or II. The remaining showed no new thrombotic formation or stenosis. (5) Of the nine grade Vb patients, five underwent coronary artery bypass grafting. The youngest of these patients, a 22 months old girl, died intraoperatively. The remaining four recovered postoperatively and were followed up for 8 to 90 months. One patient had a preoperative left ventricular ejection fraction (LVEF) of only 32.8%, with LVEF remaining abnormal post-CABG, between 35% and 44%. The remaining three patients had normal heart size, cardiac function, and electrocardiogram.Of the other four grade Vb patients, two were contraindicated for surgery due to severe heart failure and loss of myocardial activity. Two other cases are being followed up closely due to their young age of 9 months. CONCLUSION: Coronary angiography is safe and efficacious in children, and even in infants.It is the current gold standard tool for grading Kawasaki coronary artery disease. Proper anticoagulation therapy can markedly decrease the incidence of coronary artery occlusion in patients with Kawasaki coronary artery disease. Safe ranges of corrected INR should be between 1.5 and 2.5 after taking warfarin. CABG is an effective treatment for severe coronary artery disease with myocardial ischemia.

Assessment of cardiac output and volume load by transpulmonary thermodilution technique in immature pigs.

OBJECTIVE: To assess the accuracy of cardiac output (CO) measured by transpulmonary thermodilution technique (TPTD)and explore the validity of intrathoracic blood volume index (ITBVI) for assessment of circulatory volume status. METHODS: Ten immature pigs with a mean weight of (20.6±1.9)kg were studied during the conditions including normovolemia, hypervolemia, and hypovolemia. Simultaneous CO was measured in each condition using pulmonary artery thermodilution (PATD) method and TPTD. More specifically, CO (COPA) was determined with PATD, while CO (COTP) and ITBVI were determined with TPTD. All measurements were repeated 3 times. Central venous pressure (CVP) and heart rate were measured at the same time. The potential correlations of CVP and ITBVI with cardiac index (CI) and stroke volume index (SVI) in each blood volume status were analyzed. RESULTS: A total of 90 simultaneous measurements of COPA and COTP in 3 different blood volume conditions were made. The correlation coefficient between the two measurements was 0.977 (P<0.001) and the mean difference was (0.25±0.26)L/min (95%CI:0.20-0.30 L/min, P<0.001). The coefficient of variation of COTP was 3.7%, while COPA was 5.4%. Compared with those in normovolemia, CVP and ITBVI in hypervolemia significantly increased (P=0.002, 0.019), ITBVI in hypovolemia decreased significantly (P<0.001), and CVP in hypovolemia decreased insignificantly (P=0.05). Correlation analysis revealed a significant correlation between ITBVI with CI and SVI in normovolemia (r=0.741, P=0.014; r=0.885, P=0.001). In contrast, correlations between CVP with CI and SVI were poor. CONCLUSIONS: TPTD can accurately and precisely measure CO in different blood volume conditions. ITBVI measured by TPTD has better validity for the assessment of circulatory volume status than CVP.

Pulse oximetry with clinical assessment to screen for congenital heart disease in neonates in China: a prospective study.

BACKGROUND: Several pioneering studies have provided evidence for the introduction of universal pulse oximetry screening for critical congenital heart disease. However, whether the benefits of screening reported in studies from high-income countries would translate with similar success to low-income countries is unknown. We assessed the feasibility and reliability of pulse oximetry plus clinical assessment for detection of major congenital heart disease, especially critical congenital heart disease, in China. METHODS: We did a pilot study at three hospitals in Shanghai to assess the accuracy of pulse oximetry plus clinical assessment for detection of congenital heart disease. We made a data collection plan before recruitment. We then undertook a large, prospective, and multicentre screening study in which we screened all consecutive newborn babies (aged 6-72 h) born at 18 hospitals in China between Aug 1, 2011, and Nov 30, 2012. Newborn babies with positive screen results (either an abnormal pulse oximetry or abnormal clinical assessment) were referred for echocardiography within 24 h of screening. We identified false-negative results by clinical follow-up and parents' feedback. We calculated sensitivity, specificity, positive and negative predictive values, and positive and negative likelihood ratios for pulse oximetry alone, and in combination with clinical assessment, for detection of major and critical congenital heart disease. FINDINGS: In the pilot study, 6785 consecutive newborn babies were screened; 46 of 49 (94%) cases of asymptomatic major congenital heart disease and eight of eight (100%) cases of asymptomatic critical disease were detected by pulse oximetry and clinical assessment. In the prospective multicentre study, we screened 122,738 consecutive newborn babies (120,707 asymptomatic and 2031 symptomatic), and detected congenital heart disease in 1071 (157 critical and 330 major). In asymptomatic newborn babies, the sensitivity of pulse oximetry plus clinical assessment was 93·2% (95% CI 87·9-96·2) for critical congenital heart disease and 90·2% (86·4-93·0) for major disease. The addition of pulse oximetry to clinical assessment improved sensitivity for detection of critical congenital heart disease from 77·4% (95% CI 70·0-83·4) to 93·2% (87·9-96·2). The false-positive rate for detection of critical disease was 2·7% (3298 of 120,392) for clinical assessment alone and 0·3% (394 of 120,561) for pulse oximetry alone. INTERPRETATION: Pulse oximetry plus clinical assessment is feasible and reliable for the detection of major congenital heart disease in newborn babies in China. This simple and accurate combined method should be used in maternity hospitals to screen for congenital heart disease. FUNDING: Key Clinical Research Project sponsored by Ministry of Health, Shanghai Public Health Three-Year Action Plan sponsored by Shanghai Municipal Government, and National Basic Research Project of China.

Torsional mechanics of the left ventricle in patients after surgical repair of tetralogy of Fallot.

BACKGROUND: This study aimed to test the hypothesis that alteration of left ventricular (LV) torsional mechanics occurs in patients after repair of tetralogy of Fallot (TOF) and is associated with right ventricular (RV) volume overload and changes in LV configuration. METHODS AND RESULTS: Fifty-five TOF patients aged 19.0 ± 8.1 years and 27 age-matched healthy controls were studied. The LV and RV volumes were measured using 3-dimensional echocardiography while LV geometry was quantified by the diastolic eccentricity index (EI). The LV peak systolic torsion and systolic twisting and diastolic untwisting velocities were determined by speckle tracking. Compared with controls, patients had significantly greater RV end-systolic (P < 0.001) and diastolic (P < 0.001) volumes and LV diastolic EI (P < 0.001). In contrast, LV peak apical rotation (P < 0.001), systolic torsion (P = 0.004), systolic twisting velocity (P = 0.001), and diastolic untwisting velocity (P = 0.001) were lower in patients than in controls. For the whole cohort, RV EDV and LV diastolic EI correlated negatively with peak systolic torsion, systolic twisting velocity, and diastolic untwisting velocity (all P ≤ 0.001). Systolic torsion correlated strongly with diastolic untwisting velocity (r = 0.72, P < 0.001), while systolic twisting velocity correlated with LV ejection fraction (r = 0.3, P = 0.005). CONCLUSIONS: LV torsional mechanics is impaired and is negatively related to RV volume overload and LV eccentricity in patients after TOF repair.

Atypical Shone's complex diagnosed by echocardiography.

Shone's complex is a rare and severe type of congenital left-sided obstructive lesion of multiple cardiovascular levels. This report aims to present the authors' experience diagnosing atypical Shone's complex using echocardiography. Atypical Shone's complex was diagnosed for 38 consecutive patients (18 boys and 20 girls, ages 1 day to 15 years) using echocardiography. All the diagnoses were confirmed by cardioangiography, surgery, or both. Among the congenital left-sided obstructive lesions, four levels of obstruction coexisted in 3 cases (7.9%), three levels in 10 cases (26.3%), and two levels in 25 cases (65.8%). Supra-annular mitral stenosis caused by a supravalvular mitral ring was diagnosed in 1 case. Mitral valvular stenosis was diagnosed in 24 cases including a parachute mitral valve (4 cases), a double-orifice mitral valve (1 case), and partly fused thickened mitral valvular leaflets (19 cases). Subaortic stenosis was diagnosed in 10 cases including circumferential membranous subaortic stenosis (1 case), tunnel-type subaortic narrowing (2 cases), and asymmetric obstructive fibrous ridge below the aortic valve (7 cases). Aortic stenosis was diagnosed in 25 cases including the bicuspid aortic valve (12 cases), the hypoplasia aortic annulus (3 cases), and partly fused or thickened tri-leaflet aortic valves (10 cases). Supra-aortic stenosis was diagnosed in 6 cases including the localized type (4 cases) and the diffused type (2 cases). Coarctation of the aorta was diagnosed in 26 cases. Of these, focal coarctation was described in 15 cases, long segment coarctation in 7 cases, and dysplastic isthmus and/or descending aorta in 4 cases. Secondary changes in Shone's complex manifested as left atrial dilation (26 cases), left ventricular hypertrophy (26 cases), and pulmonary hypertension (19 cases). Echocardiography plays an important role in the diagnosis of atypical Shone's complex, in comprehensive evaluation of the number, position, morphology, and severity of left-sided obstructions.

Diastolic ventricular interaction in patients after atrial switch for transposition of the great arteries: a speckle tracking echocardiographic study.

BACKGROUND: We tested the hypothesis that diastolic ventricular interaction occurs after atrial switch operation for transposition of the great arteries (TGA) and that subpulmonary LV diastolic function is influenced by septal geometry. METHODS: Twenty-nine patients (male 19) after atrial switch operation for TGA aged 20.8 ± 4.1 years and 27 healthy controls were studied. Two-dimensional longitudinal systolic strain, systolic (SRs), early diastolic (SRe), and late diastolic (SRa) strain rates of both ventricles were determined using speckle tracking echocardiography. Early diastolic trans-atrioventricular velocity (E) and myocardial early diastolic myocardial velocity (e) at the ventricular free wall-annular junction were measured. Geometry of the morphologic left ventricle was quantified by the diastolic eccentricity index (EI). RESULTS: In both systemic and subpulmonary ventricles, SRe and SRa were significantly lower and trans-atrioventricular E/e ratios higher in patients than controls (all p<0.001). In patients, RV SRe correlated with left ventricular (LV) SRe (r=0.49, p=0.008), and RV SRa correlated with LV SRa (r=0.46, p=0.01). Significant leftward shifting of the septum in patients was reflected by the greater LV EI (p<0.001). In patients, LV EI correlated with age- and sex-adjusted z score of LV end-diastolic volume. As a group, LV EI correlated negatively with LV SRe (r=-0.62, p<0.001) and LV SRa (r=-0.51, p<0.001), and positively with mitral E/e ratio (r=0.33, p=0.02). CONCLUSIONS: Systemic RV diastolic dysfunction occurs after atrial switch operation and correlates with subpulmonary LV diastolic dysfunction. The observed diastolic ventricular interaction may potentially be mediated through alteration of septal geometry.

Restrictive right ventricular physiology and right ventricular fibrosis as assessed by cardiac magnetic resonance and exercise capacity after biventricular repair of pulmonary atresia and intact ventricular septum.

BACKGROUND: The hypertrophic myocardium, myocardial fiber disarray, and endocardial fibroelastosis in pulmonary atresia and intact ventricular septum (PAIVS) may provide anatomic substrates for restrictive filling of the right ventricle. HYPOTHESIS: Restrictive right ventricle (RV) physiology is related to RV fibrosis and exercise capacity in patients after biventricular repair of PAIVS. METHODS: A total of 27 patients, age 16.5 +/- 5.6 years, were recruited after biventricular repair of PAIVS. Restrictive RV physiology was defined by the presence of antegrade diastolic pulmonary flow and RV fibrosis assessed by late gadolinium enhancement (LGE) cardiac magnetic resonance. Their RV function was compared with that of 27 healthy controls and related to RV LGE score and exercise capacity. RESULTS: Compared with controls, PAIVS patients had lower tricuspid annular systolic and early diastolic velocities, RV global longitudinal systolic strain, systolic strain rate, and early and late diastolic strain rates (all P < 0.05). A total of 22 (81%, 95% confidence interval: 62%-94%) PAIVS patients demonstrated restrictive RV physiology. Compared to those without restrictive RV physiology (n = 5), these 22 patients had lower RV global systolic strain, lower RV systolic and early diastolic strain rates, higher RV LGE score, and a greater percent of predicted maximum oxygen consumption (all P < 0.05). CONCLUSION: Restrictive RV physiology reflects RV diastolic dysfunction and is associated with more severe RV fibrosis but better exercise capacity in patients after biventricular repair of PAIVS.

Myocardial deformation in patients with Beta-thalassemia major: a speckle tracking echocardiographic study.

BACKGROUND: Increasing data suggest that parameters of myocardial deformation are strong indices of ventricular systolic and diastolic function. We sought to determine myocardial deformation of the left ventricle and assess relationship of deformation rates with myocardial iron load in patients with beta-thalassemia major. METHODS: The left ventricular longitudinal, circumferential, and radial myocardial deformation was determined using speckle tracking echocardiography in 42 thalassemia patients aged 24.4 +/- 6.4 years. The results were compared with those of 38 age-matched controls. The rates of longitudinal and circumferential deformation were correlated with cardiac T2* magnetic resonance findings. RESULTS: Compared with controls, patients had significantly greater global systolic radial strain (P = 0.001), but similar global systolic longitudinal (P = 0.12) and circumferential strain (P = 0.84). On the other hand, patients had significantly lower longitudinal systolic strain rate (SR) (P = 0.019), longitudinal early diastolic SR (P = 0.036), and circumferential early diastolic SR (P = 0.04) than controls. The cardiac T2* findings correlated positively with longitudinal (r = 0.44, P = 0.004) and circumferential early diastolic SR (r = 0.37, P = 0.019), but not with the respective systolic SRs and left ventricular ejection fraction (all P > 0.05). Patients with iron overload (T2*< 20 msec), compared to those without, had significantly lower longitudinal (1.45 +/- 0.33/sec vs. 1.76 +/- 0.27/sec, P = 0.002) and circumferential (1.01 +/- 0.31/sec vs. 1.22 +/- 0.31/sec, P = 0.03) early diastolic SR. CONCLUSIONS: Patients with beta-thalassemia major have reduced longitudinal systolic SR, longitudinal early diastolic SR, and circumferential early diastolic SR. The rates of diastolic deformation in the longitudinal and circumferential dimensions are inversely related to myocardial iron overload.