Journal of Pediatric Cardiology and Cardiac Surgery
Online ISSN : 2433-1783
Print ISSN : 2433-2720
Volume 3, Issue 1
Displaying 1-11 of 11 articles from this issue
Original Articles
  • Masataka Kitano, Hisashi Sugiyama, Kenji Baba, Hirotaka Ohki, Koichi K ...
    2019 Volume 3 Issue 1 Pages 1-7
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    Background: Although ductal stenting (DS) has been performed in patients with severe congenital heart disease in Japan, the frequency of DS performed in Japan remains unclear; moreover, no stent has been approved for arterial duct (AD) under Japanese health insurance (JHI).

    Methods: The Health Insurance Committee of the Japanese Society of Pediatric Interventional Cardiology conducted a questionnaire-based survey to investigate the current status of DS performed and its necessity in Japan and evaluated the efficacy and safety of the procedure to seek approval for its use under JHI.

    Results: DS was performed in 64 cases at 12 institutions between April 2013 and March 2016. A total of 62 DS were performed for restoring the systemic circulation in patients with hypoplastic left heart syndrome, subaortic stenosis with interrupted aortic arch, and others. The three major indications for performing DS were high risk of alternative surgery such as the Norwood procedure, urgent procedures owing to the closure of AD, and based on own institutional principle. Complete success was achieved in all the 62 procedures, and prostaglandin E1 was withdrawn in 60 cases (97%). Major complications included stent migration/embolization in 6 cases (9.7%), 4 of which were replaced using a balloon, and dissection in 3 cases (4.8%). Mortality related to the procedures or complications was absent. Majority of the patients reached the next stage surgery.

    Conclusions: The present study strongly recommends that both balloon-expandable and self-expandable stents must be approved for the safe and effective ductal intervention under JHI as soon as possible.

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  • Yoonjin Kang, Jae Gun Kwak, Woong-Han Kim
    2019 Volume 3 Issue 1 Pages 8-13
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    Background: Surgical intervention to treat patent ductus arteriosus (PDA) is required when pharmacological treatment is contra-indicated or fails; however, the optimal time to perform surgery remains unclear. We evaluated the clinical outcome of surgical closure of PDA on symptomatic preterm neonates according to the timing of the operation.

    Methods: We retrospectively evaluated 117 symptomatic preterm neonates who underwent surgical correction of PDA between April 2010 and December 2016.

    Results: Morbidity and mortality rates were compared based on the timing of surgery. The early occlusion group was associated with significantly lower incidences of bronchopulmonary dysplasia (BPD) (odds ratio [OR] 0.298, p=0.011) and pneumonia (OR 0.874, p=0.023) than the late occlusion group. However, the former group had higher rates of mortality and intraventricular hemorrhage (IVH). Early occlusion was performed principally on neonates with primary occlusions. The morbidity rate of the secondary occlusion group did not vary by surgical timing.

    Conclusions: Delayed PDA closure after medical treatment failure in neonates was associated with a higher incidence of BPD. Early primary PDA closure may improve the respiratory outcomes of preterm neonates, with acceptable safety.

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  • Hideharu Oka, Masaya Sugimoto, Aya Kajihama, Kouichi Nakau, Sadahiro N ...
    2019 Volume 3 Issue 1 Pages 14-19
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    Background: Measuring ventricular volume accurately is important. We attempted to estimate the feasibility of computed tomography (CT) and magnetic resonance imaging (MRI) for measuring ventricular volume in patients with congenital heart disease (CHD).

    Methods: Four different cardiac models of CHD: hearts with atrial septal defects, ventricular septal defects, tetralogy of Fallot, and single left ventricle were used. Each model was created using a three-dimensional printer. The ventricular volume of each model was measured using cardiac catheterization (Cath), CT, and MRI.

    Results: The mean percentages and standard deviations of ventricular volume that were measured with Cath, CT, and MRI were 94% (±11%), 137% (±8%), and 111% (±8%), respectively. The value of the CT group was significantly higher than that of the Cath and MRI groups (p<0.01). We also found that the variance of the measured values was small for MRI and CT. The corrected measured value of CT and MRI especially had less error than that of Cath (Cath: 103% [±16%], CT: 100% [±6%], MRI: 101% [±7%]).

    Conclusions: CT and MRI may replace Cath to evaluate the ventricular volume of children and adults with CHD.

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  • Hideharu Oka, Kouichi Nakau, Aya Kajihama, Hiroshi Azuma
    2019 Volume 3 Issue 1 Pages 20-27
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    Background: The crochetage pattern is an important finding in atrial septal defect (ASD); however, the reason for this pattern is unclear. We hypothesized that the crochetage pattern is related to the right ventricle stretch direction.

    Methods: Stretch values were calculated in eight ASD patients with the crochetage pattern, eight ASD patients without the crochetage pattern, and eight controls who underwent cardiac catheterization. The right ventricle systolic area was subtracted from the right ventricle diastolic area in posteroranterior (PA) and left lateral views on right ventricle angiography. The area was divided into three parts at the position of the tricuspid valve and adjusted according to body surface area.

    Results: ASD patients with the crochetage pattern did not have significantly higher pulmonary/systemic blood flow ratios or right ventricle end diastolic volume indices than those without the crochetage pattern. Stretch values towards the left were higher in ASD patients with the crochetage pattern than in controls. Stretch values in the lower direction in the PA view were higher in ASD patients with the crochetage pattern than in those without the crochetage pattern. Multiple regression analysis revealed that stretch values in the lower direction in the PA view influenced the number of crochetage patterns.

    Conclusion: ASD patients with the crochetage pattern have significantly higher stretch values in the lower and left directions in the PA view than those without the crochetage pattern and controls. Stretch values in the lower direction of the right ventricle are associated with the crochetage pattern in ASD patients.

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  • Masayuki Abiko, Tesshu Otagiri, Tatsunori Takahashi, Makoto Sato, Seig ...
    2019 Volume 3 Issue 1 Pages 28-34
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    Cor triatriatum dexter (CTD) is a very rare cardiac anomaly in which a membrane divides the right atrium (RA) into two chambers. Clinical presentation of CTD is slightly variable. Most patients are asymptomatic, and a lesion is discovered during echocardiography or surgery for other cardiac lesions. When accompanied with interatrial defects, patients with CTD exhibit cyanosis because of the right-to-left shunt via interatrial communication. Moreover, CTD and cyanotic congenital heart diseases require differentiation. To our knowledge, there are no reports of infants with a hypoplastic RV who were diagnosed with CTD, and there is no consistent opinion about the best treatment strategy for CTD. Here, we describe a male neonate with CTD and a hypoplastic RV having symptoms of central cyanosis soon after birth. A large membranous structure was identified in the RA and flow from the inferior vena cava was partially redirected into the left atrium (LA) via the patent foramen ovale (FO). We surgically resected a large membranous structure from the RA and partially closed the FO when the patient was 4 months old. We performed cardiac catheterization 12 months after surgery and confirmed that the RV was well-developed and had good function. We speculate that for CTD with hypoplastic RV and inter-atrial communication, resection of the Eustachian valve and partial closure of the FO may be effective treatment strategies as long as the size of the RV is considered. Further studies on RV size and intervention for the FO in such cases of CTD are needed.

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Case Reports
  • Sadahiro Furui, Takaomi Minami, Mitsuru Seki, Shun Suzuki, Kensuke Oka ...
    2019 Volume 3 Issue 1 Pages 35-38
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    Congenital pulmonary vein stenosis (CPVS) is extremely rare, accounting for 0.03% of cases of congenital heart disease. The prognosis of CPVS is poor because the secondary pulmonary hypertension (PH) is progressive. Treatments options include surgical repair using a sutureless technique and stent implantation; however, the stenosis often recurs. Herein, a boy with a partial anomalous pulmonary venous connection was treated for CPVS using a sutureless technique at the age of 1 year old; however, the stenosis recurred, and he experienced repeated hemoptysis from the age of 2 years and 8 months. Because of the severity of his condition, surgery or a catheter intervention would have been challenging, and the bleeding points could not be identified. After detailed investigation using cardiac catheterization and three-dimensional computed tomography, a partial resection of the lower lobe of the right lung was performed, and hemoptysis was resolved. Compensatory enlargement of the remaining lobes maintained the patient’s respiratory function with no mediastinal shift. There was no evidence of hemoptysis during 5 years following the lobectomy, and he goes to school without exercise restrictions. Because lung transplantation is not a viable treatment option in Japan, lobectomy may become a therapeutic option for a pulmonary hemorrhage caused by restenosis of the pulmonary vein after CPVS repair using a sutureless technique.

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  • Shin Ono, Hideaki Ueda, Ki-sung Kim, Toshihide Asou
    2019 Volume 3 Issue 1 Pages 39-43
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    The prognosis in cases of right atrial isomerism with obstructed total anomalous pulmonary venous connection remains unsatisfactory. In particular, mortality following surgical repair of obstructed total anomalous pulmonary venous connection in the neonatal period is high. This case report describes a patient who underwent primary drug-eluting stent implantation for treatment of obstructed total anomalous pulmonary venous connection associated with right atrial isomerism. The stent was placed on the day of birth, and a definitive Fontan procedure was performed at 16 months of age. We also discuss the stent specimen, which had been implanted in the vertical vein for a total of 5 months. To our knowledge, this case report is the first to describe a drug-eluting stent specimen implanted in a draining vein associated with right atrial isomerism.

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Original Articles
  • Yusuke Yamamoto, Akinori Hirano, Yukihiro Yoshimura, Masatsugu Terada, ...
    2019 Volume 3 Issue 1 Pages 44-49
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    The importance of the short-term continuous-flow ventricular assist device (STCF-VAD) for the management of pediatric patients with refractory cardiogenic shock is growing along with the increasing use of the Berlin Heart EXCOR pediatric ventricular assist device. Nevertheless, clinical experience with the STCF-VAD for pediatric patient is insufficient with unsatisfactory outcomes to date, especially in Japan. We report our experience of one month of successful management of the STCF-VAD in a one-year-old boy of dilated cardiomyopathy. He was previously treated with VA-ECMO, and then converted to the STCF-VAD as a bridge to the EXCOR. Although six times of circuit exchanges were required due to thrombus formation, the patients experienced no complication and finally converted to the EXCOR after 29 days of STCF-VAD support. Our experience suggests that adequate antithrombotic therapy to reduce the risk of both thrombogenesis and hemorrhagic complications, frequent assessment of the circuit to detect thrombi as early as possible, and prompt circuit exchange in case of a floating or growing thrombus are the key to preventing adverse events. Application of percutaneous cannulae exclusively for the EXCOR might also be advantageous for prevention of thrombogenesis and elimination of re-open chest surgery at the transition to the EXCOR. The STCF-VAD therapy requires highly strict management, and therefore should be managed by an experienced medical team.

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  • Kensaku Matsuda, Hideki Tatewaki, Yusaku Nagatomo, Yuichiro Hirata, Ha ...
    2019 Volume 3 Issue 1 Pages 50-55
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    Anomalous origin of the right pulmonary artery from the ascending aorta (AORPA) is a rare congenital cardiac malformation. Clinical manifestations usually appear in infants or, more rarely, in newborns and include respiratory distress or congestive heart failure due to increased pulmonary resistance. Primary total correction of AORPA reportedly can result in excellent survival with a low incidence of reintervention. We report here two cases of staged repair of AORPA. Case 1 involved a 25-day-old girl who had a complicating respiratory syncytial (RS) virus infection. RS virus infection in children with congenital heart disease is associated with high mortality and morbidity, and cardiac surgery performed with cardiopulmonary bypass during symptomatic RS virus infection is associated with a high risk of postoperative complications, especially postoperative pulmonary hypertension. Therefore, we decided that the initial palliation should consist of right pulmonary artery banding, and total correction was achieved 2 months later. Case 2 involved an almost 2-month-old girl who initially underwent right pulmonary artery banding due to severe pulmonary hypertension, and total correction was achieved 13 days later. Both patients were discharged in good condition without any clinical symptoms. us, right pulmonary artery banding appears to be a good surgical option for patients with AORPA and complicated condition.

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  • Shintaro Terashita, Hideyuki Nakaoka, Keijiro Ibuki, Sayaka Ozawa, Kei ...
    2019 Volume 3 Issue 1 Pages 56-60
    Published: January 31, 2019
    Released on J-STAGE: March 18, 2021
    JOURNAL FREE ACCESS

    Williams-Beuren syndrome (WBS) is caused by microdeletions of 7q11.23, including the ELN gene, and the characteristic vascular lesions include supravalvular aortic stenosis (SVAS) and peripheral pulmonary artery stenosis (PPS). We identified a patient who has the characteristic cardiovascular lesions of WBS but does not have the 7q11.23 deletion: analysis of ELN identified a nonsense mutation. This case is a baby girl, whose cardiovascular abnormalities were not identified during fetal life. She was admitted with systemic cyanosis after birth, and was diagnosed with SVAS and supravalvular pulmonary stenosis (SVPS). At 10 months of age she underwent cardiac catheterization and confirmed SVAS, which was accompanied by a thin ascending aorta, PPS narrowing from hilar, and SVPS. Due to high blood pressure and hyperreninemia, she was diagnosed with renal vascular hypertension caused by bilateral renal artery stenosis identified on MDCT and began an oral carvedilol regimen. Mental retardation and malformations associated with WBS were not observed and a FISH analysis excluded a microdeletion encompassing ELN. Since mutations in ELN have been identified in cases of isolated SVAS it was screened and a novel nonsense mutation in Exon 24 was identified. Thus, the cardiovascular lesions were considered to be caused by elastin deficiency due to this ELN mutation. We suggest that if a patient has the characteristic cardiovascular lesions of WBS, but is FISH negative, then ELN should be screened for mutations.

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