Keywords: conotruncal anomaly, subarterial ventricular septal defect, balloon pulmonary valvuloplasty, tetralogy of Fallot, infant 藤岡 泰生 1) 金 成海 1) 満下 紀恵 1) 石垣 瑞彦 1) 松尾久実代 1) 佐藤 慶介 1) 芳本 潤 1) 新居 正基 1) 坂本喜三郎 2) 小野 安生 1) 静岡県立こども病院循環器科 1) ,心臓血管外科 2) Background: Conotruncal anomalies can cause severe cyanosis that requires intervention in the neonatal and early infantile period. Objectives: To evaluate the effectiveness and outcomes of using balloon pulmonary valvuloplasty (BVP) as an initial palliative procedure in such cases, particularly in those with subarterial ventricular septal defects. Materials and Methods: Between August 2002 and December 2012, 9 infants (8 males and 1 female) with conotruncal anomalies (3 with tetralogy of Fallot, 4 with double outlet right ventricle, and 2 with congenitally corrected transposition of the great arteries) underwent BVP. Echocardiographic and catheterization data, as well as the patientsʼ clinical course, were retrospectively examined. Results: The mean balloon to annulus ratio (B/A ratio) was 1.08 ± 0.15. Moreover, the mean systemic oxygen saturation increased from 75% ± 6% before BVP to 87% ± 7% after BVP (p <0.001) ; however, no significant changes in peak velocity and peak pressure gradient at the right ventricular outflow tract were noted, and only minimal pulmonary regurgitation occurred. All the patients, except for 1 with tracheomalacia, were discharged without the need for home oxygen therapy. One patient subsequently required pulmonary arterial banding due to pulmonary over-circulation. No deaths were noted, and definitive repair was achieved in all the patients. Conclusion: As an initial palliative procedure, BVP involving a relatively smaller balloon size is less invasive, and provides appropriate pulmonary blood flow and bridge to the definitive surgery. 要 旨 背景:円錐動脈幹異常では新生児期や乳児期早期に重度のチアノーゼを呈し,治療介入を要することがある. 目的:流出路心室中隔欠損を伴い肺動脈弁狭窄が主体の本疾患群における,初回姑息治療としての経皮的肺動脈弁 バルーン拡大術 (BVP) の有効性と予後を検討すること. 対象:2002 年 8 月~ 2012 年 12 月に当院にて新生児期・乳児期早期に BVP を行った 9 例 〔男:女 = 8:1,内訳: Fallot 四徴症 (TOF) 3,両大血管右室起始 (DORV) 4,修正大血管転位 (ccTGA) 2〕 . 方法:心エコーによる肺動脈弁輪径から,BVP 時のバルーン径 / 肺動脈弁輪径比 (B/A ratio) を算出.BVP 前後の SpO2,右室流出路の最大血流速度 (Vp) ,最大圧較差 (PG) の変化と合併症の有無,さらに,最終手術までの経過を検 討した. 結果:B/A ratio は 1.08 ± 0.15 であった.BVP 後,SpO2 は 75 ± 6%から 87 ± 7%に上昇 した (p < 0.001) .Vp,PG に 2013 年 9 月 5 日受付 2014 年 1 月 28 日受理 別刷請求先:〒 420-8660 静岡県静岡市葵区漆山 860 静岡県立こども病院循環器科 藤岡 泰生 日本小児循環器学会雑誌 第30巻 第3号 42 PEDIATRIC CARDIOLOGY and CARDIAC SURGERY VOL.30 NO.3 (263-270) 原 著 有意な変化は認めず,肺動脈弁逆流は少量以下にとどまった.気管軟化症の 1 例を除き,全例酸素投与を中止し退院 できた.1 例で高肺血流に対し,肺動脈絞扼術を要した.死亡例はなく,全例最終手術に到達できた. 結語:初回姑息治療としての BVP は低侵襲であり,バルーン径を過大としないことで,適切な肺血流増加が得られ, 最終手術への有効な橋渡しとなる. 平成26年5月1日 43 264
Objective Prenatal recognition of coarctation of the aorta (CoA) may improve neonatal survival and reduce morbidity. However, prenatal diagnosis of CoA remains challenging, with relatively high false–positive and false–negative rates. This study aimed to identify a novel formula based on fetal echocardiographic measures to predict prenatal identification of CoA. Methods A retrospective comparison on the echocardiographic evaluation of 30 patients with suspected CoA between May 2016 and April 2021 was performed. Fetal echocardiograms were measured for the following: Z-score of right and left ventricular diameters, tricuspid and mitral valve diameters, pulmonary artery and ascending aorta diameters, pulmonary valve and aortic valve diameters, ductus arteriosus and aortic isthmus diameters and their respective ratios, and distal aortic arch (DA) index, which is the distance between the second and third branches of the aortic arch / distal aortic arch diameter. The patients were divided into a postnatal surgical intervention group (n = 13) and a non-intervention group (n = 17). Results The measurements that showed significant differences were aortic isthmus diameter Z-score (p < 0.001), ductus arteriosus diameter/aortic isthmus diameter (p < 0.001) and DA index (p < 0.001). In the receiver operating characteristic curves analysis, the DA index was the largest with an area under the curve of 0.941 and a cutoff value of 1.28, with a sensitivity of 85% and a specificity of 94%. Among the three parameters that showed significant differences, the DA index had the lowest false-positive rate (5.9%). Conclusions The DA index ≧ 1.28 allows for the extraction of fetal CoA cases requiring surgical intervention.
An isolated left common carotid artery is very rare, and only 13 cases have been reported thus far. All those cases were accompanied by a right aortic arch and aberrant left subclavian artery, and the connecting vessel between the pulmonary artery and left common carotid artery was thought to be ductal tissue. However, there have been no reports that have followed the natural closure of this vessel. We present a case in whom we could observe the closing process of this vessel at the connection between the left common carotid artery and main pulmonary artery in association with a tetralogy of Fallot.
Plastic bronchitis (PB) is a rare but severe complication in patients following the Fontan operation, the final palliative procedure for single ventricle heart disease. PB is characterised by the formation of rubbery casts of the tracheobronchial tree that may cause cough, wheezing, dyspnoea and hypoxia. Progressive airway obstruction may lead to asphyxia and death, and the overall mortality rate is 15.2%. Most patients receive a combination therapy from three different treatment strategies, that is, therapy for relief of airway obstruction, anti-inflammatory treatment and treatment to improve haemodynamics of the Fontan physiology. Therapy improving haemodynamics consists of optimising the Fontan circulation or cardiac function, antiarrhythmic therapy, inhibition of lymph leakage/production, decompression of the Fontan circulation and heart transplantation.We present the case of a child with PB after extracardiac Fontan operation, who was refractory to balloon angioplasty and surgical release for pulmonary vein (PV) stenosis. We decided to perform stent implantation for the right PVs through transconduit puncture instead of fenestration because the position of fenestration was too high to access the PVs. After PV stenting via the transconduit approach, she experienced improvement in cough, wheezing and breathlessness, and a significant reduction in airway cast formation.
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