Sudden infantile death due to left ventricular myocardial ischemia associated with undiagnosed single coronary artery of Lipton's type LII-B.

Single coronary artery (SCA) is a rare malformation, particularly in isolation without other congenital heart defects. Here, we describe a case of sudden infantile death due to myocardial ischemia associated with undiagnosed SCA. A 1-year-and-7-month-old female infant died suddenly after vomiting at home. A medicolegal autopsy revealed that the orifice of the right coronary artery (RCA) was absent. As expected, the left coronary artery was detached from the left coronary sinus; however, the ostium was covered with a flap-like fibrous ridge. The thin RCA originated from the left main trunk (LMT) and passed between the aorta and pulmonary artery trunk. The left circumflex artery branched from the LMT at a right angle. Judging from the origin and course of the anomalous RCA, the deceased was diagnosed with SCA of Lipton's type LII-B. Microscopic examination revealed ischemic changes around the endocardium of the lateral wall of the left ventricle (LV) rather than on the side of the abnormal RCA. This evoked attention, as there appeared to be some discrepancy. To the best of our knowledge, this is the first case report describing sudden infantile death related to LV myocardial ischemia associated with undiagnosed SCA of Lipton's type LⅡ-B.

Observation of Atrial Fibrillation Dependent on an Intra-atrial Re-entrant Tachycardia Within the Right Atrium in a Repaired Tetralogy of Fallot.

Atrial fibrillation (AF) can occur predominantly associated with right atrial (RA) lesions in congenital heart disease, particularly when the RA cavity is dilated. RA electrical potentials occasionally appear organized during AF. We clearly mapped such areas circumscribed by an intra-atrial re-entrant circuit during an isoproterenol infusion, in a patient with a repaired tetralogy of Fallot, using an ultrahigh-density mapping system and its beat acceptance criteria function. Ablation of areas inside the re-entrant circuit successfully eliminated the AF. Our experience indicated that a macro-re-entrant tachycardia was a driver as well as a trigger of AF of this right-sided origin.

Chez les patients atteints d'une cardiopathie congénitale, la fibrillation auriculaire (FA) peut souvent survenir en association avec des lésions auriculaires droites (AD), en particulier lorsque la cavité AD est dilatée. Lors d'une FA, il peut arriver que les potentiels électriques AD semblent normaux. Chez un patient ayant une tétralogie de Fallot réparée, nous avons clairement cartographié des zones délimitées par un circuit de réentrée intra-auriculaire lors d'une perfusion d'isoprotérénol, et ce, à l'aide d'un système de cartographie à très haute densité et de ses critères d'acceptation liés aux battements cardiaques. L'ablation des régions se trouvant dans le circuit de réentrée a permis d'éliminer la FA avec succès. Notre expérience a démontré qu'une tachycardie macroréentrante avait été un facteur déterminant et même un déclencheur de la FA, laquelle est apparue à droite.

Neonatal repair of atypical double outlet right ventricle.

BACKGROUND: A complex and rare form of double outlet right ventricle needs careful attention when choosing the optimal strategy for repair. AIM OF THE STUDY: To point out retrospectively what could have been done differently in our unique patient. METHODS: Primary repair was arranged in a neonate with double outlet right ventricle (of a non-committed ventricular septal defect type and lack of the outlet septum between the semilunar valves) with right aortic arch and dextro-malposition of great arteries. RESULTS: We managed to achieve intraventricular rerouting via a right ventricular incision concomitantly with the arterial switch maneuver. The patient is doing well with an excellent hemodynamic status. CONCLUSIONS: We considered that the radical approach we chose appeared to be sensible in this particular patient, although some other options could have been available.

Perspective of preexcitation induced cardiomyopathy; early septal contraction, and subsequent rebound stretch.

BACKGROUND: Preexcitation-induced cardiomyopathy (PIC) is defined as a disease presenting ventricular dyssynchrony because of preexcitation through an accessory pathway (AP), being a cousin of pacing-induced cardiomyopathy. The present review aims at providing perspective of this uncharted subgroup. METHODS: In order to determine mechanisms and clinical characteristics of PIC, 63 patients in 29 literature reports were reviewed. RESULTS: A median age at onset was 4 (0.1-59) years; 55 patients (87%) under 18 years old including 16 infants. Twenty patients (32%) experienced supraventricular tachycardia prior and subsequent to the PIC onset. Heart failure and left ventricular (LV) dysfunction did not correlate with other clinical features. All the 65 APs identified (duplicated in 2 patients) were located on the right side of the atrioventricular junction; at the septal area (in 55%) or the anterolateral aspect (in 22%). AP conduction was successfully eliminated by medical or interventional treatments where attempted. LV function returned to normal within 6 months in 67% of patients, while recovery took longer than 3 years in 8%. Frequently seen at the basal segments of the interventricular septum were early contraction within the QRS complex, dyskinesis at mid-systole, and aneurysm/bulging or local thinning. CONCLUSIONS: Several characteristic factors became clear as described above. Rebound stretch following early shortening of the interventricular septum is seemingly the major mechanism of PIC, and thus a right septal or right anterior/anterolateral AP needs attention as a higher risk for PIC.

Use of hydrocolloid dressing in infants requiring open chest management after cardiac surgery.

PURPOSE: Sternal splintage is known as an effective maneuver to stabilize hemodynamics during the immediate postoperative period, particularly in very sick infants. On the other hand, its wound management is not always straightforward. We employed dressing using a product made of a hydrocolloid material in such circumstances. This report describes our experience in utilizing the dressing in term of its potential advantages. MATERIALS AND METHODS: Six infants needed open chest management following complicated procedures for congenital heart disease. A polytetrafluoroethylene patch was fixed to augment the skin defect at the time of sternal splintage, and a hydrocolloid dressing was applied to entirely cover the wound including the suture line. RESULT: All patients survived their difficult circumstances. None of them suffered wound complications such as infection or healing problem during sternal splintage or subsequent to eventual chest closure. The dressing product was easy to handle with no adverse events associated with its material. CONCLUSIONS: It is reconfirmed that a dressing made of hydrocolloid material was of practical use for sealing the wound in infants requiring open chest management after cardiac surgery.

Cor Triatriatum Can Coexist With Common Pulmonary Venous Atresia.

Surgical treatment was very urgently carried out in an extremely cyanotic newborn with common pulmonary venous atresia. The diagnosis was confirmed during the procedure, and the common venous chamber was anastomosed to the left atrium through a transverse incision onto the right and the left atria. Unfortunately, pulmonary hypertension persisted during the postoperative course and even got worse gradually. Computed tomography eventually illustrated residual cor triatriatum. The initial atriotomy had been entirely above the diaphragm within the left atrium. Consequently, the abnormal structure had been overlooked. The patient is doing well 1 month after the surgical revision. Pulmonary hypertension disappeared.

A retrospective study of factors associated with persistent delirium.

BACKGROUND: It has been reported that delirium causes various problems. Many researchers have reported the risk factors associated with the onset of delirium; however, there are few reports focused on persistent delirium. This study aimed to identify the risk factors associated with persistent delirium. METHODS: A total of 573 patients hospitalised in Nara Prefecture General Medical Centre from October 2014 through September 2017 who were referred to the psychiatry consultation service were included in this study. Persistent delirium was defined as delirium lasting for 14 days or more. A retrospective study was carried out based on the patients' records. The relationship between various background factors and persistent delirium was statistically analysed. RESULTS: Of the 573 hospitalised patients, 295 were diagnosed as having delirium. Forty-six patients with persistent delirium and 181 patients with nonpersistent delirium were included in this study. Multivariable logistic regression analyses revealed that male gender, opioid analgesics use, non-opioid analgesics use, and low serum sodium were significantly and independently associated with persistent delirium. Ramelteon or trazodone was used significantly more in persistent delirium, although each use was not significant. CONCLUSION: This is the first study to reveal that male gender and use of analgesics were associated with persistent delirium in general hospital. However, as this is a case-control study and may contain bias, future cohort studies and intervention studies are needed. It is also necessary to investigate the relevance of the 'degree of pain' behind the use of analgesics.

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.

CRT in a congenital heart disease patient with interventricular dyssynchrony due to an RV conduction delay.

We encountered a unique pattern of cardiac dyssynchrony in a patient with complex congenital heart disease (heterotaxy syndrome) with a biventricular physiology and systemic left ventricle (LV). On speckle tracking echocardiography, dyssynchrony was not detected within the LV, but was noted in an interventricular fashion between the LV and right ventricle (RV). An electrophysiologic study revealed a conduction delay in the subpulmonary RV. Cardiac resynchronization therapy provided reverse cardiac remodeling and an excellent result in our patient by placing the pacing leads around the dyssynchronous lesion.

Is two-staged repair for truncus arteriosus type A3 unpractical?

BACKGROUND: A complex and rare form of persistent truncus arteriosus needs careful attention when choosing the optimal strategy for repair. AIM OF THE STUDY: We herein describe our surgical strategy of a small infant having this malformation concomitantly with right aortic arch, unusual pulmonary artery branching and a left superior vena cava. METHODS: The patient underwent initially bilateral pulmonary arterial banding followed by the Rastelli type definitive repair. The pulmonary arteries were unified in front of the left superior vena cava, and the right ventricular outflow tract was readily reconstructed. RESULTS: The patient is doing well with an excellent hemodynamic status. CONCLUSIONS: We considered the two-stage approach was sensible in this particular patient.

Preoperative simulation for complex transposition of great arteries using a three-dimensional model.

We utilised a cast preoperatively prepared on the basis of the patient's computed tomography data to clarify unusual orientation of the great arteries (transposition of great arteries, coarctation of the aorta, and abnormally branching central pulmonary arteries) in a 2.2-kg neonate. The three-dimensional model was very useful for designing the surgical procedure - being easy to handle for real and precise simulation of the reparative manoeuvres.

Major adverse events and atrial tachycardia in Ebstein's anomaly predicted by cardiovascular magnetic resonance.

OBJECTIVES: Patients with Ebstein's anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes. METHODS: Seventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.4 (range 0.4-10.9) years for clinical outcomes, namely major adverse cardiovascular events (MACEs: sustained ventricular tachycardia/heart failure hospital admission/cardiac transplantation/death) and first-onset atrial tachyarrhythmia (AT). RESULTS: CMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06, 95% CI 1.168 to 3.623, p=0.012 and HR 2.35, 95% CI 1.348 to 4.082, p=0.003, respectively), LV stroke volume index (HR 2.82, 95% CI 1.212 to 7.092, p=0.028) and cardiac index (HR 1.71, 95% CI 1.002 to 1.366, p=0.037); all remained significant when tested solely for mortality. History of AT (HR 11.16, 95% CI 1.30 to 95.81, p=0.028) and New York Heart Association class >2 (HR 7.66, 95% CI 1.54 to 38.20, p=0.013) were also associated with MACE; AT preceded all but one MACE, suggesting its potential role as an early marker of adverse outcome (p=0.011).CMR variables associated with first-onset AT (n=17; 21.5%) included RVEF (HR 1.55, 95% CI 1.103 to 2.160, p=0.011), total R/L volume index (HR 1.18, 95% CI 1.06 to 1.32, p=0.002), RV/LV end diastolic volume ratio (HR 1.55, 95% CI 1.14 to 2.10, p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03, 95% CI 1.00 to 1.07, p=0.041); the latter two combined enhanced risk prediction (HR 6.12, 95% CI 1.67 to 22.56, p=0.007). CONCLUSION: CMR-derived indices carry prognostic information regarding MACE and first-onset AT among adults with unrepaired EA. CMR may be included in the periodic surveillance of these patients.

Persistent fifth aortic arch: the "great pretender" in clinical practice.

Persistence of the embryonic "fifth aortic arch" in postnatal life is a rare, enigmatic - and at times controversial - condition, with variable anatomical forms and physiological consequences. First described in humans over 40 years ago by Van Praagh, the condition was labelled the "great pretender" by Gerlis 25 years later, because of its apparent propensity to mimic anatomically similar structures. Despite many subsequent case reports citing the condition, the true developmental origin of these structures remains unresolved, and has been the subject of debate among embryologists for more than a century. A persistent fifth aortic arch has been defined as an extrapericardial structure, arising from the ascending aorta opposite or proximal to the brachiocephalic artery, and terminating in the dorsal aorta or pulmonary arteries via a persistently patent arterial duct. This description may therefore encompass various anatomical forms, such as a unilateral double-lumen aortic arch, an unrestrictive aortopulmonary shunt, or a critical vascular channel for either the systemic or pulmonary circulation. The physiological properties of these vessels, such as their response to prostaglandins, may also be unpredictable. In this article, we demonstrate a number of cases that fulfil the contemporary definition of "persistent fifth aortic arch" while acknowledging the embryological controversies associated with this term. We also outline the key diagnostic features, particularly with respect to the use of new cross-sectional imaging techniques.

Intracardiac anatomical relationships and potential for streaming in double inlet left ventricles.

The aim of this study was to gain better understanding of the variable anatomical features of double inlet left ventricle hearts without cavopulmonary connection that would potentially facilitate favorable streaming. Thirty-nine post-mortem specimens of double inlet left ventricle without cavopulmonary connection were investigated. The focus was on anatomical characteristics that could influence the flow and separation of deoxygenated and oxygenated blood in the ventricles. Elements of interest were the ventriculoarterial connection, the spatial relationship of the ventricles, the position and size of the great arteries, the ventricular septal defect, the presence of relative outflow tract stenosis and the relationship of the inflow and outflow tracts. The most common anatomy was a discordant ventriculoarterial connection with an anatomically left-sided morphologically right ventricle (n = 12, 31%). When looking at the pulmonary trunk/aorta ratio, 21 (72%) hearts showed no pulmonary stenosis relative to the aorta. The ventricular septal defect created a relative subpulmonary or subaortic stenosis in 13 (41%) cases. Sixteen (41%) hearts had a parallel relationship of the inflow and outflow tracts, facilitating separation of deoxygenated and oxygenated blood streams. On the other end of the spectrum were 10 (25%) hearts with a perpendicular relationship, which might lead to maximum mixing of the blood streams. The relationship of the inflow and outflow tracts as well as the presence of (sub-) pulmonary stenosis might play a crucial role in the distribution of blood in double inlet left ventricle hearts. Additional in vivo studies will be necessary to confirm this postulation.

Immediate and Midterm Cardiac Remodeling After Surgical Pulmonary Valve Replacement in Adults With Repaired Tetralogy of Fallot: A Prospective Cardiovascular Magnetic Resonance and Clinical Study.

BACKGROUND: Pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot provides symptomatic benefit and right ventricular (RV) volume reduction. However, data on the rate of ventricular structural and functional adaptation are scarce. We aimed to assess immediate and midterm post-PVR changes and predictors of reverse remoeling. METHODS: Fifty-seven patients with repaired tetralogy of Fallot (age ≥16 y; mean age, 35.8±10.1 y; 38 male) undergoing PVR were prospectively recruited for cardiovascular magnetic resonance performed before PVR (pPVR), immediately after PVR (median, 6 d), and midterm after PVR (mPVR; median, 3 y). RESULTS: There were immediate and midterm reductions in indexed RV end-diastolic volumes and RV end-systolic volumes (RVESVi) (indexed RV end-diastolic volume pPVR versus immediately after PVR versus mPVR, 156.1±41.9 versus 104.9±28.4 versus 104.2±34.4 mL/m2; RVESVi pPVR versus immediately after PVR versus mPVR, 74.9±26.2 versus 57.4±22.7 versus 50.5±21.7 mL/m2; P<0.01). Normal postoperative diastolic and systolic RV volumes (the primary end point) achieved in 70% of patients were predicted by a preoperative indexed RV end-diastolic volume ≤158 mL/m2 and RVESVi ≤82 mL/m2. RVESVi showed a progressive decrease from baseline to immediate to midterm follow-up, indicating ongoing intrinsic RV functional improvement after PVR. Left ventricular ejection fraction improved (pPVR versus mPVR, 59.4±7.6% versus 61.9±6.8%; P<0.01), and right atrial reverse remodeling occurred (pPVR versus mPVR, 15.2±3.4 versus 13.8±3.6 cm2/m2; P<0.01). Larger preoperative RV outflow tract scar was associated with a smaller improvement in post-PVR RV/left ventricular ejection fraction. RV ejection fraction and peak oxygen uptake predicted mortality (P=0.03) over a median of 9.5 years of follow-up. CONCLUSIONS: Significant right heart structural reverse remodeling takes place immediately after PVR, followed by a continuing process of further biological remodeling manifested by further reduction in RVESVi. PVR before RVESVi reaches 82 mL/m2 confers optimal chances of normalization of RV function.

Contemporary cardiac surgery for adults with congenital heart disease.

OBJECTIVE: Advances in early management of congenital heart disease (CHD) have led to an exponential growth in adults with CHD (ACHD). Many of these patients require cardiac surgery. This study sought to examine outcome and its predictors for ACHD cardiac surgery. METHODS: This is an observational cohort study of prospectively collected data on 1090 consecutive adult patients with CHD, undergoing 1130 cardiac operations for CHD at the Royal Brompton Hospital between 2002 and 2011. Early mortality was the primary outcome measure. Midterm to longer-term survival, cumulative incidence of reoperation, other interventions and/or new-onset arrhythmia were secondary outcome measures. Predictors of early/total mortality were identified. RESULTS: Age at surgery was 35±15 years, 53% male, 52.3% were in New York Heart Association (NYHA) class I, 37.2% in class II and 10.4% in class III/IV. Early mortality was 1.77% with independent predictors NYHA class ≥ III, tricuspid annular plane systolic excursion (TAPSE) <15 mm and female gender. Over a mean follow-up of 2.8±2.6 years, 46 patients died. Baseline predictors of total mortality were NYHA class ≥ III, TAPSE <15 mm and non-elective surgery. The number of sternotomies was not independently associated with neither early nor total mortality. At 10 years, probability of survival was 94%. NYHA class among survivors was significantly improved, compared with baseline. CONCLUSIONS: Contemporary cardiac surgery for ACHD performed at a single, tertiary reference centre with a multidisciplinary approach is associated with low mortality and improved functional status. Also, our findings emphasise the point that surgery should not be delayed because of reluctance to reoperate only.

Right Ventricular Pressure Overload and Pathophysiology of Growing Porcine Biomodel.

The primary objective was to create a clinically relevant model of right ventricular hypertension and to study right ventricular myocardial pathophysiology in growing organism. The secondary objective was to analyse the effect of oral enoximone (phosphodiesterase inhibitor) therapy on right ventricular haemodynamic parameters and myocardial changes in biomodel of right ventricular hypertension. The study included a total of 12 piglets of 42 days of age. Under general anaesthesia, pulmonary artery banding (PAB) was performed surgically to constrict the main pulmonary artery to about 70-80 % of its original dimension. The study presented two groups of animals labelled C (control animals with PAB; n = 8) and E (animals with PAB and oral administration of enoximone; n = 4). Direct pressure and echocardiographic measurements were taken during operation (time-1), and again at 40 days after surgery (time-2). The animals were killed, and tissue samples from the heart chambers were collected for quantitative morphological assessment. Statistical analysis was performed on all acquired data. At time-2, the median weight of animals doubled and the median systolic pressure gradient across the PAB increased (46.59 ± 15.87 mmHg vs. 20.29 ± 5.76 mmHg; p < 0.001). Changes in haemodynamic parameters were compatible with right ventricular diastolic dysfunction in all the animals. Apoptosis, tissue proliferation and fibrosis were identified in all the myocardial tissue samples. Right ventricular pressure overload leads to increased apoptosis of cardiac myocytes, proliferation and myocardial fibrosis. Our study did not show evidence of haemodynamic benefit or myocardial protective effect of oral enoximone treatment.

Surgical aspects of atrial arrhythmia : Right atrial ablation and anti-arrhythmic surgery in congenital heart disease.

BACKGROUND: Atrial arrhythmias are frequently described in congenital heart disease. OBJECTIVES: To provide a surgical perspective of anti-arrhythmic procedures and strategic approaches. METHODS: Discussion of the history of anti-arrhythmic treatments in congenital heart disease. RESULTS: Before the advent of the Maze procedure (first published in 1991), surgery mainly focused on patients with Wolff-Parkinson-White syndrome and also on arrhythmias in Ebstein's malformation. Atrial septal defects (ASD) subsequently received more attention, i.e., in terms of atrial arrhythmia in the natural prognosis and the surgically modified course and in terms of surgical versus transcatheter approaches. Based on the background of various atrial arrhythmia mechanisms of ASD, several surgical procedures have been reported, ranging from the bilateral full Maze procedure to simple modification of right atriotomy. The so-called right atrial Maze procedure occupies a special position from the viewpoint of cardiology in acquired heart disease, especially in cases of frequently occurring right heart failure. In hearts with more complex structural abnormalities, a detailed understanding of the conditions to improve overall surgical outcome and develop future "anti-arrhythmic" strategies is necessary. CONCLUSIONS: It is important to precisely specify factors in the individual cases, not only morphological diversity but also technical and strategic variations and their consequences. A variety of anti-arrhythmic surgical procedures are currently available. How and when to use which procedure requires professional insight and cautious clinical decision-making.

The Importance of Walking for Control of Blood Pressure: Proof Using a Telemedicine System.

BACKGROUND: Regular physical activity (PA), including daily walking, reduces the risk of many chronic diseases, especially hypertension. Pedometer is a potential motivational aid for increasing PA. In the present study, we used a telemedicine system and analyzed the relationship between daily walking, calculated by pedometers, and blood pressure (BP). METHODS: BP was measured at home twice a day (morning and evening) using an oscillometric automatic device. Body weight (BW) and percent body fat (%BF) were measured after BP measurement. Daily walking steps (DWS) were calculated by a pedometer. These daily parameters were transmitted through the Internet to a central server computer and sent to the Medical Health Center. RESULTS: Sixty-nine (N = 69) hypertensive patients were included in this study. The mean follow-up period was 378 days. Electronic data from a pedometer (DWS) were associated with reduced BW, body mass index, and %BF. Hypertensive patients were divided into two groups based on the DWS. In the high DWS group, morning systolic BP and diastolic BP and evening systolic BP were reduced after induction of the telemedicine system. CONCLUSION: A telemedicine system confirmed the usefulness of walking to control BP in hypertensive patients.