A geometrical pitfall of Area-Length method; -Is left ventricle volume evaluation of repaired Tetralogy of Fallot by angiocardiography accurate?

Biplane Area-Length (AL) method by left ventriculography (LVG) has been widely adopted as a standard method to estimate left ventricular volume. However, we have experienced difficulties in adopting the value by AL method for the children with Tetralogy of Fallot (TOF) due to the discrepancy among volumetric modalities. This study validated some limitations of AL method, considering the basic principles of its formulation. A single center retrospective cohort study was conducted for 1 year. The confirmed 22 cases with repaired TOF at our hospital were enrolled. The clinical characteristics, some cardiac MRI analyses, and all the cardiac catheterization studies were collected. Angiographic data were compared with historic cohorts of Kawasaki disease without any coronary artery lesions by using AL method. Cardiac MRI analyses of ten TOF patients were additionally available. LVG studies showed that the length of the long axis on anteroposterior view (AP) was not equal to that on lateral view (LT) due to anatomically apical elevation in TOF, followed by a significant difference found in the sagittal lengths of the LV long axis between AP and LT (P = 0.003). Because the difference critically affected the formula depending on biplane AL method, the calculated LVEDV of TOF group appeared overestimated, compared with the control group (TOF vs control group: 119.5% ± 6.3% vs 96.4 ± 3.5% of Normal, P = 0.006). Available cardiac MRI analyses of some patients in TOF group revealed 55% increase of LVEDV by AL method (angiocardiography 116 ± 7.0 vs CMR 75 ± 3.7 ml/m2, P = 0.0025). A pitfall exists when applying biplane AL method to measure LV volume especially for TOF patients, because the long axis on AP view is not always identical to that on LT view.

Left ventricular inflow obstruction due to a coronary arteriovenous fistula: a paediatric case report.

BACKGROUND: We report a rare case of left ventricular inflow obstruction from a branch of the left circumflex coronary artery to the right atrium caused by a coronary arteriovenous fistula (CAVF) in a young Japanese male child. CASE PRESENTATION: The patient was diagnosed with CAVF following a heart murmur shortly after birth. The left-to-right shunt caused right ventricular volume overload and pulmonary congestion. An emergency surgical intervention was performed for the CAVF on day 6 after birth. However, by 5 years of age, his left ventricular inflow obstruction worsened. We found an abnormal blood vessel originating from the proximal part of a branch of the left circumflex coronary artery, circling the outside of the mitral valve annulus along the medial side of the coronary sinus. As the child gets older, the blood inflow into the left ventricle might get restricted further, resulting in left-sided heart failure. CONCLUSION: Our findings suggest that even after CAVF closure surgery, it is essential to monitor for complications caused by progressive dilatation of a persistent CAVF.

Pulmonary vasodilators can lead to various complications in pulmonary "arterial" hypertension associated with congenital heart disease.

Congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH) is one of the major complications in patients with CHD. A timely closure of the left-to-right shunt will generally result in the normalization of the pulmonary hemodynamics, but a few patients have severe prognosis in their early childhood. We hypothesized that wide-ranging pathological mechanism in PAH could elucidate the clinical state of severe CHD-PAH. Using electronic medical records, we retrospectively analyzed six infants with severe CHD-PAH who had treatment-resistant PH. All patients were born with congenital malformation syndrome. After starting on a pulmonary vasodilator, five of the six patients developed complications including pulmonary edema and interstitial lung disease (ILD), and four patients had alveolar hemorrhage. After steroid therapy, the clinical condition improved in four patients, but two patients died. The autopsy findings in one of the deceased patients indicated the presence of recurrent alveolar hemorrhage, pulmonary venous hypertension, ILD, and PAH. Based on the clinical course of these CHD-PAH in patients and the literature, CHD-PAH can occur with pulmonary vascular obstructive disease (PVOD)/pulmonary capillary hemangiomatosis (PCH), ILD, and/or alveolar hemorrhage. The severity of CHD-PAH may depend on a genetic disorder, respiratory infection, and upper airway stenosis. Additionally, pulmonary vasodilators may be involved in the development of PVOD/PCH and ILD. When patients with CHD-PAH show unexpected deterioration, clinicians should consider complications associated with PVOD/PCH and/or pulmonary disease. In addition, the choice of upfront combination therapy for pediatric patients with CHD-PAH should be selected carefully.

Potential Value of Native T1 Mapping in Symptomatic Adults with Congenital Heart Disease: A Preliminary Study of 3.0 Tesla Cardiac Magnetic Resonance Imaging.

The native T1 value at 3.0 Tesla is a sensitive marker of diffuse myocardial damage. We evaluated the clinical usefulness of native T1 mapping in symptomatic adults with congenital heart disease (CHD), particularly in the systemic right ventricle (RV). Prospectively, 45 consecutive symptomatic adults with CHD were enrolled: 20 with systemic RV and 25 with tetralogy of Fallot underwent cardiac magnetic resonance (CMR) imaging at 3.0 Tesla. The Modified Look-Locker Inversion recovery sequence was used for T1 mapping. Cardiovascular events in the systemic RV were defined as heart failure and tachyarrhythmia. Brain natriuretic peptide (BNP) and indexed systemic ventricular end-diastolic volume were significantly higher in the systemic RV group. The native T1 value and extracellular volume (ECV) of the septal and lateral walls were higher in the systemic RV group, suggesting high impairment of the myocardium in the systemic RV group. There was a strong correlation between the native T1 value and ECV of the septum (r = 0.58, P = 0.03) and lateral wall (r = 0.56, P = 0.046) in the systemic RV group. Seven patients with systemic RV had cardiovascular events. In univariate logistic regression analysis, BNP and native T1 values of the insertion point were important for predicting cardiovascular events. The native T1 value at 3.0 Tesla may be a sensitive, contrast-free, and non-invasive adjunct marker of myocardial damage in CHD and predictive of cardiovascular events in the systemic RV.

Inter- and intra-ventricular dyssynchrony in the systemic right ventricle is a surrogate marker of major cardiac events in mildly symptomatic patients.

The aim of the study was to evaluate systemic right ventricular (RV) dyssynchrony in patients with congenitally corrected transposition of the great arteries (CCTGA) and transposition of the great arteries (TGA) with New York Heart Association functional class (NYHA FC) < III. We used cardiac magnetic resonance (CMR) to evaluate the dyssynchrony and assessed whether RV dyssynchrony can be predictive of major cardiac events in their early stages in these patients. We enrolled 71 consecutive, NYHA FC < III patients with systemic RV who underwent CMR between April 1995 and December 2016. We measured intra- and inter-ventricular dyssynchrony using a feature-tracking method of cine magnetic resonance imaging. The predictors of major cardiac events were analyzed using the Cox hazard analysis. The data from 36 patients with CCTGA and 35 patients with TGA after an atrial switch were analyzed. Seven (19.4%) patients with CCTGA and 6 (17.1%) patients with TGA showed a QRS duration of ≥ 130 ms. There were significant intra- and inter-dyssynchrony in the systemic RV groups, compared to healthy controls. The average follow-up period was 5.1 ± 3.9 years. From among patients with CCTGA, 9 (25.0%) had major cardiac events. The parameters including NYHA FC, indexed RV volume, longitudinal early diastolic strain rate, and intra- and inter-ventricular dyssynchrony were predictive of major cardiac events. From among patients with TGA, 12 (34.3%) had major cardiac events. Age, NYHA FC, QRS duration, RV volume, RV mass index, LV volume, global longitudinal/circumferential strain and intraventricular dyssynchrony, were all predictive of major cardiac events. Systemic RV in NYHA FC < III patients with CCTGA and TGA, have obvious intra- and inter-dyssynchrony, suggesting ineffective wall motion and potential RV dysfunction. Intraventricular dyssynchrony can be an adjunct predictor of major cardiac events in mildly symptomatic patients with both CCTGA and TGA.

Hypothermia, bradycardia, and hypotension during glucocorticoid or cyclosporine A therapy in a boy with Kikuchi-Fujimoto disease.

Kikuchi-Fujimoto disease (KFD) is an inflammatory disease of unknown aetiology characterised by fever and cervical lymphadenopathy. Although KFD is a self-limiting disease, patients with severe or long-lasting course require glucocorticoid therapy. We presently report a 17-year-old boy with KFD who had seven relapses since the onset at 4 years old. He suffered from hypothermia, bradycardia, and hypotension during the treatment with prednisolone or methylprednisolone. All of his vital signs recovered after cessation of the drug in addition to fluid replacement and warming. Thus, glucocorticoid was effective but could not be continued because of the adverse event. Although hypothermia developed during the treatment with 5 mg/kg/day of cyclosporine A (CsA) at his second relapse, he was successfully treated with lower-dose CsA (3 mg/kg/day). Thereafter, he had five relapses of KFD until the age of 12 years and was treated by 1.3-2.5 mg/kg/day of CsA. Hypothermia accompanied by bradycardia and hypotension developed soon after concomitant administration of ibuprofen at his fifth and sixth relapses even during low-dose CsA therapy. Conclusively, glucocorticoid, standard dose of CsA, or concomitant use of non-steroidal anti-inflammatory drugs may cause hypothermia, bradycardia, and hypotension and needs special attention. Low-dose CsA could be a choice for such cases with KFD.

Successful treatment of IgA vasculitis with prolonged cutaneous manifestation with colchicine in a 10-year-old boy.

We report a 10-year-old boy with immunoglobulin (Ig)A vasculitis (IgAV) with prolonged cutaneous manifestations who was successfully treated with colchicine. At the age of 9 years, he was diagnosed as having IgAV by typical purpura, abdominal pain, and haematochezia. Initially, his severe gastrointestinal manifestation subsided by prednisolone 60 mg/day and intravenous methylprednisolone pulse therapy. However, his gastrointestinal manifestation was glucocorticoid-dependent and refractory to factor XIII concentrate, intravenous IgG, and mycophenolate mofetil. His abdominal pain and haematochezia responded to the combination therapy with dapsone and low dose of prednisolone 5 mg/day and did not relapse even after discontinuation of dapsone. On the other hand, the effect of dapsone on his cutaneous manifestation was dose-dependent as well as dapsone had no glucocorticoid-sparing effect. Approximately 12 months after onset, colchicine treatment was started, which resulted in remission of his chronic cutaneous manifestation. After prednisolone was tapered off, his cutaneous manifestation is currently well-controlled on colchicine 0.5 mg/day without adverse events. He had never complicated kidney involvements. In conclusion, it is observed that colchicine treatment exerts a beneficial effect in IgAV patients with prolonged cutaneous manifestation refractory to multiple drugs.

A Cardiac Arrest Case due to Left Coronary Artery Compression in Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.

We report the case of an adult who had a cardiac arrest in the setting of pulmonary hypertension and a previously repaired intermediate atrioventricular septal defect, with left main coronary trunk stenosis due to dilatation of the main pulmonary artery. In patients with pulmonary hypertension exhibiting anginal symptoms, it is advisable to perform chest contrast computed tomography to confirm the pulmonary artery diameter and the presence of coronary artery compression. In addition, our case highlights the importance of early collaboration among specialists during the transition from adolescence to adulthood.

Nous décrivons le cas d'un adulte ayant subi un arrêt cardiaque alors qu'il présentait une hypertension pulmonaire et qu'il avait déjà subi la réparation d'une communication septale auriculoventriculaire intermédiaire, avec sténose de l'artère coronaire gauche principale causée par la dilatation de l'artère pulmonaire principale. Chez les patients atteints d'hypertension pulmonaire qui présentent des symptômes angineux, il est recommandé d'effectuer une tomodensitométrie thoracique avec produit de contraste pour confirmer le diamètre de l'artère pulmonaire et la présence d'une compression de l'artère coronaire. Notre cas souligne également l'importance d'établir sans tarder une collaboration entre spécialistes lors de la transition entre l'adolescence et l'âge adulte.

The relationship between extracellular volume fraction in symptomatic adults with tetralogy of Fallot and adverse cardiac events.

BACKGROUND: To investigate the relationship between the extracellular volume fraction (ECV) measured using cardiac magnetic resonance (CMR) T1 mapping and cardiac events in symptomatic adults with tetralogy of Fallot (TOF). METHODS: A total of 60 consecutive symptomatic adults (35.4±13.8 years old) with repaired TOF who were in New York Heart Association functional class 2-4 were prospectively enrolled. Native T1 values and ECV of the free walls of the right ventricle (RV), the left ventricle, and the ventricular septum were obtained by CMR T1 mapping using a 3.0T scanner and the saturation recovery method. Correlations between T1 mapping measurements, conventional parameters, and predictors of adverse cardiac events were analyzed. The patients with episodes of only atrial arrhythmia were excluded. RESULTS: Significant correlations were observed between RV functional parameters, RV-ECV, and septum-ECV. Follow-up period was 747.1±315 days and 13 patients had cardiac events: Acute heart failure and/or sustained ventricular tachycardia occurred in 8 patients and 5 patients underwent cardiac surgery. Cox-hazard analysis revealed that septum-ECV and RV-ECV were predictors of adverse events (hazard ratio of septum-ECV and RV-ECV: 1.41 and 1.19, 95% CI: 1.05-1.89, 1.004-1.41 with p-values of 0.02, and 0.045, respectively). Adults with septum-ECV >29.0% and RV ejection fraction (EF) <45.0% were more likely to experience cardiac events (log rank test: p<0.043). CONCLUSIONS: RV-ECV and septum-ECV correlate with RV functional parameters. Biventricular ECVs can be predictors of adverse cardiac events in adults with TOF. In particular, the combination of septum-ECV and RVEF was a useful predictor, compared to the use of a single CMR parameter.

Diagnostic dilemma in a case of obstructive sleep apnoea syndrome with reversible pulmonary hypertension and right heart failure: A case report.

Childhood obstructive sleep apnoea syndrome (OSAS) secondary to adenoid hyperplasia is known to give rise to pulmonary hypertension. However, we present a case of a toddler with pulmonary hypertension (PH) and right heart failure due to OSAS, the cause of which is difficult to identify. After the patient underwent an adenotonsillectomy, OSAS disappeared and the PH eventually resolved. Both paediatricians and otolaryngologists should know that paediatric OSAS can occur even in the setting of mild, clinically insignificant palatine tonsil hypertrophy and adenoid hyperplasia. Surgical intervention should be considered without losing the opportunity if it could be the cause of PH.

Advanced pathological study for definite diagnosis of mitochondrial cardiomyopathy.

AIMS: Mitochondrial cardiomyopathy (MCM) is difficult to make a definite diagnosis because of various cardiovascular phenotypes and no diagnostic criteria in the pathology examination. We aim to add myocardial pathology to the diagnostic criteria for mitochondrial respiratory chain disorders. METHODS: Quantitative analysis of mitochondria using electron microscopy and immunohistopathological analysis with respiratory chain enzyme antibodies were performed in 11 patients with hypertrophic or restrictive cardiomyopathy who underwent endomyocardial biopsy for possible MCM . Respiratory chain enzymatic assay in biopsied myocardium and genetic studies were also performed in all the subjects to define MCM. RESULTS: Four patients were diagnosed with MCM according to the recent criteria of mitochondrial respiratory chain disorders. Using electron microscopy with quantitative analysis, the volume density of mitochondria within cardiac muscle cells was significantly increased in the MCM group compared with the non-MCM group (p=0.007). Immunohistopathological results were compatible with the result of the respiratory chain enzymatic assay. CONCLUSIONS: Pathological diagnosis of MCM could be confirmed by a quantitative study of electron microscopy and immunohistopathological analysis using the mitochondrial respiratory chain enzyme subunit antibody.