Successful Truncal Valve Replacement After Truncal Valve Repairs.

The surgical management of severe truncal valvular dysfunction is still challenging in neonates with persistent truncus arteriosus. This report describes a 14-day-old neonate with severe truncal valve insufficiency successfully undergoing truncal valve repairs, and followed by valve replacement at the age of 4 years. The truncal valve was quadricuspid with two large and two small leaflets, and all leaflets had severe dysplastic and myxomatous changes. We performed leaflet extension and bicuspidization valvuloplasty for this valve. This patient obtained somatic growth for 4 years without heart failure symptoms, and safely underwent prosthetic valve replacement. This technique would be effective for truncal valve dysfunction in neonates as the life-saving and the bridging procedure to valve replacement.

Long-term results after palliative intra-cardiac repair for tetralogy of Fallot and diminutive pulmonary arteries.

BACKGROUND: In patients with tetralogy of Fallot with the diminutive pulmonary arteries, we sometimes have to give up the complete intra-cardiac repair due to insufficient growth of the pulmonary arteries. We have carried out palliative intra-cardiac repair using a fenestrated patch. METHODS: Of all 202 patients with tetralogy of Fallot in our centre since 1996, five patients (2.5%) with the diminutive pulmonary arteries underwent palliative intra-cardiac repair using a fenestrated patch. Mean operative age was 1.8 years. Previous operation was Blalock-Taussig shunt in 4. At operation, the ventricular septal defect was closed using a fenestrated patch and the right ventricular outflow tract was enlarged. Follow-up period was 9.8 ± 2.6 years. RESULTS: There were no operative and late deaths. Fenestration closed spontaneously on its own in four patients 2.7 ± 2.1 years after the intra-cardiac repair with a stable haemodynamics; however, the last patient with the smallest pulmonary artery index had supra-systemic pressure of the right ventricle post-operatively. The fenestration was emergently enlarged. Systemic arterial oxygen saturation was significantly and dramatically increased from 83.5 to 94% after the palliative intra-cardiac repair, and to 98% at the long term. A ratio of systolic pressure of the right ventricle to the left was significantly decreased to 0.76 ± 0.12 at the long term. Now all five patients were Ross classification class I. CONCLUSION: Although frequent catheter and surgical interventions were needed after the palliative intra-cardiac repair, this repair might be a choice improving quality of life with good results in patients with tetralogy of Fallot associated with the diminutive pulmonary arteries.

Evaluating the response to cardiac resynchronization therapy performed with a new ventricular morphology-based strategy for congenital heart disease.

BACKGROUND: In cardiac resynchronization therapy (CRT) for patients with congenital heart disease (CHD) and a ventricular morphology other than a systemic left ventricle (LV), we previously proposed pacing sites that are different from those used for a systemic LV. The leads should be placed laterally on opposite sides of both ventricles in patients with short-axis dyssynchrony and a single ventricular physiology with two ventricles, whereas they should be placed at the farthest sites along the longitudinal direction in the right ventricle (RV) in patients with long-axis dyssynchrony of the RV. Moreover, in patients with interventricular dyssynchrony and a biventricular physiology with a systemic RV, they should be placed at sites that both ventricles can contract simultaneously. We retrospectively investigated 27 consecutive procedures in 24 patients with CHD who underwent CRT to evaluate the effectiveness of a new ventricular morphology-based CRT strategy. The responder rate was 63% (17/27). The reasons for a non-response to CRT in 10 cases were as follows: non-optimal lead positions during CRT, 4; no systemic ventricular conduction delay or heart failure symptoms before the CRT, 5; short follow-up periods after the CRT, 2; and an extremely dilated systemic RV, 1. The responder rate became 88% (14/16), after excluding the procedures without a ventricular conduction delay or heart failure symptoms and those with non-optimal lead positions. This new strategy for CRT can provide favorable results for CHD patients with a systemic ventricular conduction delay and heart failure.

Cardiac surgical strategy for extremely low-birthweight infants with pulmonary overcirculation.

OBJECTIVES: This study aimed to review the clinical outcomes of staged cardiac surgery in extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation. METHODS: Six extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation underwent staged cardiac surgery between 2005 and 2017. The median birthweight was 895 g (range 620-990 g), and the median gestational age was 28 weeks (range 23-31 weeks). Clinical outcomes were evaluated, and we focused on pulmonary haemodynamics. RESULTS: Pulmonary artery banding or bilateral pulmonary artery banding was performed as the initial palliation at a median age of 23 days with a median body weight of 880 g. Corrective surgery was performed at a median age of 187 days with a median body weight of 3.9 kg. All of the patients successfully underwent corrective surgery and survived to date. Pulmonary hypertension regressed after corrective surgery in all of the patients, except for 1 patient with severe bronchopulmonary dysplasia. CONCLUSIONS: Acceptable outcomes can be obtained by staged cardiac surgery in extremely low-birthweight infants with congenital heart disease and pulmonary overcirculation. While early pulmonary artery banding can lead to regression of pulmonary hypertension after corrective surgery, close follow-up is required.

Long-term respiratory outcomes after primary total correction for tetralogy of Fallot and absent pulmonary valve in patient with respiratory symptoms.

OBJECTIVE: To review long-term respiratory outcomes for tetralogy of Fallot and absent pulmonary valve (TOF/APV) in respiratory symptomatic populations. METHODS: Of 25 consecutive patients undergoing primary total correction for TOF/APV between 1987 and 2016, Sixteen patients (64%) with a preoperative respiratory disturbance were enrolled. The median age at operation was 1.9 months old, including 4 neonates and 12 infants. Ten patients (62.5%) preoperatively necessitated mechanical ventilator support. During operation, dilated central pulmonary arteries (cPAs) were plicated and retracted anteriorly in all patients, except for the first patient of the study cohort. VSD was completely closed, and the right ventricular outflow tract was reconstructed with a handmade valved conduit before 1990, or a transannular patch with a handmade monocusp after 1991. RESULTS: The actuarial survival rate at 20 years was 86.7%. One patient without cPAs plication and another requiring mechanical ventilator support right after his delivery died. The median duration of postoperative mechanical ventilator support was 14 days (range, 1-183). Readmission for respiratory disturbance after discharge was frequently observed before five years of age, however, no patients were readmitted to the hospital after six years of age, during the median follow-up period of 14.7 years, with a maximum of 27.2 years. Although persistent dilatation of cPAs was common, respiratory symptoms never recurred. CONCLUSIONS: Prognostic and respiratory outcomes after primary total correction for TOF/APV in respiratory symptomatic populations were good. Successful relief of tracheal/main bronchial compression by primary total correction at the neonatal or infantile period provided delayed, but nonrecurrent improvement of respiratory disturbances.

Successful Biventricular Conversion Late After Primary One and One-Half Ventricle Repair.

A 6-year-old girl with unbalanced atrioventricular septal defect, hypoplastic right ventricle, and severe common atrioventricular valve regurgitation developed patient-prosthetic mismatch. At 6 months old, she underwent primary one and one-half ventricle repair and replacement of left side atrioventricular valve. A catheter examination showed that her right ventricular end-diastolic volume increased from 39.4 mL/m2 1 year after to 70 mL/m2 3.5 years after the previous surgery. Thus, at the timing of redo left side atrioventricular valve replacement, she was converted successfully to biventricular circulation. The postoperative course was uneventful, and the right atrial pressure was 7 mm Hg before discharge.

Importance of multidisciplinary management for pulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries and completely absent central pulmonary arteries.

BACKGROUND: To review long-term clinical outcomes for pulmonary atresia, ventricular septal defect (PA/VSD), major aorto-pulmonary collateral arteries (MAPCAs) and completely absent central pulmonary arteries (cPAs). METHODS: Of all 120 surgically treated patients with PA/VSD, MAPCAs between 1981 and 2011, 15 patients (12.5%) with completely absent cPAs were enrolled. The median age at initial surgery was 3.8 ± 4.9 years old (range 0.1-17.6) and 3.5 (2-6). Since 2003, consecutive five patients have been treated by current treatment strategy consisting of staged midline approach with percutaneous trans-catheter angioplasties (PTA), where complete unifocalization and right ventricle (RV)-PA conduit placement was performed via median sternotomy at first, then which was followed by VSD closure with one-way fenestrated patch and conduit replacement, after PTA for unifocalized MAPCAs, reconstructed cPAs, and RV-PA conduit. For patients survived after definitive repair, PTA was aggressively repeated to maintain RV systolic pressure. RESULTS: Median follow-up period was 7.8 years (0.3-21.7). Actuarial survival rate at 20 years after the initial surgical intervention was 27.5%, and all five patients treated by current treatment strategy survived to date. Of all 10 survivors after the definitive repair, serial catheter examination revealed that right to left ventricular systolic pressure ratio was maintained from 0.59 ± 0.09 (0.45-0.73) at early after the definitive repair to 0.57 ± 0.14 (0.35-0.81) at latest follow-up. CONCLUSIONS: Staged midline approach with PTA seemed feasible strategy to go on to definitive repair. Patency of MAPCAs and reconstructed cPAs were maintained by the aggressive PTA after the definitive repair.

Can Fontan Conversion for Patients Without Late Fontan Complications be Justified?

BACKGROUND: Fontan conversion from a classic Fontan operation such as atriopulmonary connection to total cavopulmonary connection with antiarrhythmia surgery is currently not indicated for patients without any late Fontan complications. METHODS: Thirty-two consecutive patients who underwent Fontan conversion between 1991 and 2012 were divided into 2 groups by the presence (group 1: n = 25, atrial tachyarrhythmia [AT] in 24 and protein-losing enteropathy in 4) or absence (group 2: n = 7) of late Fontan complications, and the surgical outcomes were retrospectively compared. During the study period, heart transplantation was not indicated for patients with failed Fontan circulation in Japan. RESULTS: The mean follow-up period was 6.2 ± 3.7 years in group 1 and 4.6 ± 3.8 years in group 2 (p = 0.29). Overall survival rate at 10 years after conversion was 71% in group 1 and 100% in group 2 (p = 0.12). Whereas preoperative AT and protein-losing enteropathy remained after conversion in 8 patients (33%) and all 4 patients (100%), respectively, in group 1, neither were observed in group 2. Cardiac catheter examinations presurgery (n = 32) at 1 year (n = 28), and at 5 years (n = 19) after the conversion showed that the cardiac index significantly and similarly improved in both groups after the conversion, and maintained for at least 5 years. CONCLUSIONS: Cardiac output similarly improved after Fontan conversion in patients with or without late Fontan complications by elimination of venous blood congestion on Fontan pathways. Although long-term follow-up is mandatory, newly onset AT was not observed after prophylactic Fontan conversion.

Staged repair of hemitruncus without prosthetic material.

Right pulmonary artery banding was performed in a patient with right hemitruncus at 17 days of age, due to severe hypertension in both pulmonary arteries and severely reduced right ventricular contraction. Following the procedure, the pulmonary hypertension and right ventricular contraction gradually improved with pulmonary vasodilator administration, and total correction was achieved two months later. A cardiac catheter examination at the 1-year follow-up showed normal pressure in both pulmonary arteries and good right ventricular contraction.

Interventricular dyssynchrony in a patient with a biventricular physiology and a systemic right ventricle.

We describe of a unique pattern of cardiac dyssynchrony in a patient with a biventricular physiology and systemic right ventricle (RV): the interventricular dyssynchrony due to a contraction delay between the left ventricle and RV. In the present case, the cineangiography and intracardiac electrocardiography of the RV did not reveal intraventricular dyssynchrony of the RV, but revealed interventricular dyssynchrony. In addition, the pressure curves of the ventricles exhibited time phase differences between the two ventricles. The cardiac resynchronization therapy determining the pacing lead positions based on the idea of interventricular dyssynchrony induced reverse cardiac remodeling in this patient with systemic RV dysfunction.

Early Single Institutional Experience of Berlin Heart EXCOR® Pediatric Ventricular Assist Device in Japan.

BACKGROUND: Since August 2015, the pediatric ventricular assist device (VAD), Berlin Heart EXCOR®, has been accepted for use in Japan.Methods and Results:Between August 2015 and July 2016, 4 pediatric patients with endstage heart failure underwent LVAD implantation with the EXCOR®device. The median age and body weight at operation were 8 months and 4.8 kg. During a median follow-up of 7.3 months (range, 5.0-10.3), all patients survived. Two patients went on to heart transplantation and the remaining 2 are on a waiting list with stable hemodynamics. CONCLUSIONS: The early outcomes of the Berlin Heart EXCOR®pediatric VAD were satisfactory. (Circ J 2016; 80: 2552-2554).

Early clinical outcomes of right ventricular outflow tract reconstruction with small caliber bovine jugular vein conduit (Contegra®) in small children.

This study reviewed early clinical outcomes of right ventricular outflow tract reconstruction with Contegra® valved conduits in pediatric patients. Between April 2013 and July 2014, thirteen pediatric patients underwent right ventricular outflow tract reconstruction with Contegra valved conduits. The size of the implanted conduits were 12 mm in 5 patients, 14 mm in 3, 16 mm in 3, and then 2 patients were implanted with bicuspidized conduits for downsizing the conduit to 9 and 10 mm in each. Follow-ups were completed in all patients. One conduit was explanted 7 days after a neonatal biventricular repair for Ebstein's anomaly and pulmonary atresia, timed to be at the point of conversion to a single ventricular palliation. Among the 5 patients who developed significant pulmonary insufficiency and/or conduit stenosis, 3 patients exhibited persistent pulmonary hypertension. Both the bicuspidized conduits resulted in early pulmonary insufficiency. One patient implanted with a ring-supported conduit developed coronary artery stenosis, caused by suppression between the ring of the implanted conduit and the annulus of the mechanical vale. Although the small caliber Contegra valved conduit might be an alternative to RVOT reconstruction, the indication should be carefully considered. More than mild pulmonary hypertension, and low body weight at operation of less than 3.0 kg caused early conduit dysfunction.

High Incidence of Dilated Cardiomyopathy After Right Ventricular Inlet Pacing in Patients With Congenital Complete Atrioventricular Block.

BACKGROUND: Some patients with congenital complete atrioventricular block (CCAVB) develop dilated cardiomyopathy (DCM) after pacemaker implantation (PMI). We evaluated the relationship between pacing site and DCM incidence. METHODS AND RESULTS: We retrospectively evaluated 38 patients with CCAVB; 8 (25%) of 32 patients who had PMI developed DCM/heart failure death (HFD) after PMI, although none of the 6 patients without PMI showed DCM/HFD. All DCM/HFD occurred within 50 months of PMI. Among the 32 patients with PMI, the DCM/HFD incidence was 55% (6/11) for right ventricular inlet (RVI), 18% (2/11) for RV apex (RVA), and 0% for left ventricle (LV) (P=0.013). At the endpoint, the LV ejection fraction and septal-to-posterior wall motion delay of patients with LV pacing were better than those for patients with other pacing sites. Among the 8 DCM/HFD patients, 2 in whom the pacing site was changed from RVI to LV apex or in whom therapy was upgraded to cardiac resynchronization remained alive with no heart failure symptoms, whereas the other 6 died of heart failure. CONCLUSIONS: A total of 25% of the patients who underwent PMI because of CCAVB, but none in the non-PMI group, developed DCM/HFD. DCM/HFD incidence was higher in patients with RVI pacing. Ventricular dyssynchrony related to pacing site may be one cause of DCM in patients with CCAVB. (Circ J 2016; 80: 1251-1258).

Steroids Improve Hemodynamics in Infants With Adrenal Insufficiency After Cardiac Surgery.

OBJECTIVE: To investigate whether steroid replacement therapy improved hemodynamics in infants after surgery for congenital heart disease only when they develop adrenal insufficiency. The authors retrospectively investigated adrenal function and evaluated hemodynamic responses to steroid replacement therapy in infants after surgery for congenital heart disease. DESIGN: Retrospective, cohort study. SETTING: Intensive care unit in the National Cerebral and Cardiovascular Center Hospital in Japan. PATIENTS: Thirty-two neonates and infants<3 months old who underwent cardiovascular surgery. INTERVENTIONS: The patients were divided into 2 groups based on corticotropin stimulation test results: group AI with adrenal insufficiency (baseline cortisol<15 µg/dL or incremental increase after testing of<9 µg/dL, with baseline cortisol of 15-34 µg/dL); and group N with normal adrenal function. The corticotropin stimulation test was performed by injecting 3.5 µg/kg of tetracosactide acetate. Hydrocortisone (1 mg/kg) was administered every 6 hours, and hemodynamics were compared before and after steroid administration between the groups. MEASUREMENTS AND MAIN RESULTS: Seven patients were classified into group AI, and demonstrated a mean blood pressure increase from 53±8 mmHg before treatment to 68±9 mmHg 18 hours after steroid administration (p<0.01). Urine output also increased, from 2.7±1.0 mL/kg/h to 4.8±1.9 mL/kg/h (p<0.05). In group N, neither mean blood pressure nor urine output increased after steroid administration. CONCLUSIONS: After surgery for congenital heart disease, one-fifth of infants developed adrenal insufficiency. Steroid replacement therapy improved hemodynamics only in the subgroup with adrenal insufficiency.

Application of Cone Reconstruction for Neonatal Ebstein Anomaly or Tricuspid Valve Dysplasia.

BACKGROUND: Outcomes of surgical intervention for severe tricuspid regurgitation related to Ebstein anomaly or tricuspid valve dysplasia in the neonatal period, particularly when associated with pulmonary atresia, are extremely poor. However, owing to emerging innovative surgical techniques, such as cone reconstruction, outcomes of tricuspid valve plasty in the neonatal period have gradually improved. METHODS: The study retrospectively reviewed the medical records of 12 neonates who were diagnosed with severe tricuspid regurgitation and pulmonary atresia related to Ebstein anomaly (n = 9) or isolated tricuspid valve dysplasia (n = 3) between 2000 and 2013. RESULTS: The first 6 patients underwent palliative therapy in anticipation of future functional single-ventricle palliation (Starnes operation). Biventricular repair was performed in the 6 patients born after 2012. As tricuspid valve plasty, cone reconstruction has been applied since 2013. Five patients underwent a Starnes operation, and 5 patients underwent biventricular repair, including 4 cone reconstructions. Four of the 5 patients who underwent a Starnes operation died in-hospital; the remaining patient underwent a Fontan operation at age 2 years. Three of the 5 patients who underwent biventricular repair survived. On echocardiogram, the 3 survivors who were treated with biventricular repair had a preoperative tricuspid regurgitation flow velocity greater than 3.0 m/s. CONCLUSIONS: Biventricular repair with cone reconstruction can be applied for severe tricuspid regurgitation due to neonatal Ebstein anomaly or tricuspid valve dysplasia with associated pulmonary atresia. A tricuspid regurgitation flow velocity greater than 3.0 m/s may be an indicator of successful biventricular repair.

Optimal pacing sites for cardiac resynchronization therapy for patients with a systemic right ventricle with or without a rudimentary left ventricle.

AIMS: This study aims to assess the impact of pacing sites on the effectiveness of cardiac resynchronization therapy (CRT) in systemic right ventricle (sRV) patients with/without a rudimentary left ventricle (rLV). METHODS AND RESULTS: We evaluated 13 procedures in 11 sRV patients with a wide QRS (>150 ms). Based on the digitalization results of ventriculography, long-axis dyssynchrony (LD) was defined as extremely delayed right ventricular (RV) outflow tract movement: ≥100 ms delay from the RV apical contraction, and short-axis dyssynchrony (SD) was defined as a paradoxical contraction between the rLV and sRV caused by a conduction delay between the two ventricles. During the follow-up period (2.1 ± 1.9 years), the response rates were 71% (5/7) and 33% (2/6) in the sRV patients with and without an rLV, respectively (P = ns). Following the CRT, the QRS duration remained similar between the responders and nonresponders. Among five responders with an rLV, the leads were placed in the longitudinal RV direction in two with LD, longitudinal RV direction with fusion of the intrinsic QRS in two with LD + SD, and laterally on opposite sides of both ventricles in one with SD. Among two responders without an rLV, the leads were placed in the longitudinal RV direction in those two with LD. CONCLUSIONS: In sRV patients with LD with/without an rLV, the leads should be placed at furthest sites in the longitudinal RV direction. In patients with an rLV and SD, the leads should be placed laterally on opposite sides of both ventricles.

Clinical Features and Surgical Outcomes of Coronary Sinus Orifice Atresia.

This study aimed to review clinical features and surgical outcomes of coronary sinus orifice atresia (CSOA). From 2003 to 2015, 6 patients were diagnosed with CSOA. Median age at diagnosis was 2 years (range 0.2-73). CSOA was preoperatively diagnosed in 2 patients, intraoperatively in 3, and postoperatively in 1. Coronary venous blood flow drained into innominate vein through the left superior vena cava (LSVC) in 2, into the atrium through Thebesian veins in 2, and into both in 2. Three patients required surgical treatment. The first patient with a congenitally corrected transposition of great arteries had undergone ligation of the LSVC and a cutback of the proximal LSVC into the functional right atrium during the double-switch operation. The second patient with tricuspid atresia gradually developed hypoxia after the Fontan operation due to the increase in veno-venous shunt blood flow from the Fontan pathway to the right atrium through the LSVC and Thebesian veins, so that the LSVC was ligated and the coronary sinus was unroofed 6 years after Fontan operation. The other functional single ventricle patient had undergone ligation of the LSVC and a cutback of the proximal LSVC into the left atrium during the Fontan operation. There were no mortalities at the median follow-up of 9 years (1-11). CSOA should be suspected when the LSVC and usual-sized innominate vein were presented. Surgical treatment is required if the postoperative hemodynamics would affect coronary venous perfusion, like single ventricular palliation (244 words).

Ductal stenting using side-branch cell dilation for aortic coarctation in high-risk patients with hypoplastic left heart syndrome.

For high-risk neonates with hypoplastic left heart syndrome (HLHS) undergoing Norwood operation, the strategy of bilateral pulmonary artery banding and ductal stenting is risky in case of coarctation of the aorta (CoA), often resulting in death. Therefore, we devised a new method of ductal stenting with side-branch cell dilation, which could overcome the constriction of the ductal arch with CoA in two HLHS patients. This is the first report that presents this method and the results. © 2015 Wiley Periodicals, Inc.

Efficacy of "Awake ECMO" for critical respiratory failure after pediatric open-heart surgery.

A 4-year-old boy with atrioventricular discordance, double-outlet right ventricle, pulmonary stenosis, and mitral regurgitation, was undergoing anatomical repair consisting of Senning, Rastelli, Damus-Kaye-Stansel procedures, and a mitral valve repair, complained of post-operative excessive airway tract secretion, which ultimately developed into acute respiratory distress syndrome (ARDS) 28 days after the operation. The cause of the ARDS was thought to be frequent manual positive pressure recruitment and prolonged inhalation of pure oxygen. At 45 days after the operation, hypercapnia and respiratory acidosis turned out to be irreversible, and therefore, veno-arterial extracorporeal membrane oxygenation (ECMO) was established utilizing the Endumo(®)4000 system. Pulmonic interstitial inflammation gradually improved while resting the lung under ECMO support; however, effective ventilation volume decreased critically because a massive pulmonary hemorrhage occurred at 2 and 9 days after the initiation of ECMO. To maximize the effectiveness of respiratory physical therapy, "Awake ECMO" was started and tidal volume dramatically increased with a regained cough reflex. Five days later, he was successfully weaned off from ECMO, and discharged 7 months after the operation without any neurological and physiological sequelae.