Prenatal Diagnosis of Ventriculocoronary Communication in a Fetus with Pulmonary Atresia with Intact Ventricular Septum : a Case Report

純型肺動脈閉鎖（pulmonary atresia with intact ventricular septum：以下PAIVS）の治療方針の決定には右室の大きさ，肺動脈の閉鎖様式に加え，冠動脈が右室内腔と繋がる異常交通（類洞交通）の有無や程度が重要とされる．今回胎児期に類洞交通を評価できたPAIVSの一例を経験したので報告する． 超音波検査にて胎児の右室が小さいことを指摘され妊娠23週に紹介となった．初診時の超音波検査にてPAIVSと診断した．また右室心尖部に心筋を貫通する両方向性血流を認め，類洞交通の存在が疑われた．その後冠動脈を起始部から系統的に描出することで，左冠動脈前下行枝からの大きな類洞交通であることを確認した．右室が高度の低形成でかつ大きな類洞交通があることから単心室修復が必要となることが予想された．児は出生後，心臓超音波検査，心臓血管造影にて同様の診断がなされ，単心室修復に至った． 胎児超音波で冠動脈を起始部から系統的に観察することで，類洞交通の評価が可能となりそれにより，出生前から治療方針の検討ができ，家族への説明に有用であった．Abnormal coronary connections between the ventricles and aorta can have significant impacts on myocardial perfusion and consequently on the approach for surgical intervention．Presence of ventriculocoronary communication（VCC），or ventriculo-coronary fistula，may be critical to peri- and postpartum management．We report the prenatal diagnosis of VCC associated with pulmonary atresia with intact ventricular septum（PAVIS）． A24-year-old woman was referred to our institution at 23＋3weeks’ gestation because of an abnormally small right ventricle（RV）in the fetus．Fetal echocardiography showed a small RV with trivial diastolic filling flow，an intact ventricular septum，and a hypoplastic pulmonary trunk with retrograde perfusion through the ductus arteriosus．Color and pulsed Doppler sonography revealed bidirectional flow at the right ventricular apex，which was confirmed as large VCC between the left anterior descending（LAD）coronary artery and RV by delineating coronary arteries from the aortic origin with color Doppler sonography． A3，070g male infant was vaginally delivered at 40＋1 weeks’ gestation．Postnatal echocardiography，computed tomography and angiography confirmed PAIVS and large VCC between the LAD coronary artery and RV．A BIalock-Taussig shunt was placed on postnatal day 11．Subsequently，a Glenn shunt was inserted at the age of 13 months，and Fontan procedure was performed at the age of 3 years． In summary，we accurately diagnosed fetal PAIVS and VCC accurately by meticulous echocardiography．Thus，we could provide appropriate counseling to parents and optimize peri- and postpartum management

A case of placenta percreta with massive hemorrhage during cesarean section

We describe a case of a 39-year-old woman diagnosed with placenta percreta complicated by massive hemorrhage during a cesarean section. At 27 weeks of gestation, she underwent an emergency cesarean section under general anesthesia for vaginal bleeding and an intrauterine infection. Soon after delivery, a massive hemorrhage was encountered while attempting to separate the placenta percreta from the bladder wall. Although total abdominal hysterectomy and partial cystectomy were performed, massive hemorrhaging persisted. Bleeding was finally controlled following bilateral internal iliac artery embolization. We used a cell salvage device and a rapid infuser for hemodynamics stabilization. Total blood loss was 47,000 mL, and anesthesia time was 12 h and 47 min. The patient was discharged on the 32nd postoperative day without major complications. Placenta accreta can be associated with life-threatening hemorrhage and it is vital to plan accordingly preoperatively

ニンシン オ ケイキ ニ ジョウミャク ケッセンショウ オ ハッショウシ センテンセイ アンチトロンビンIII ケッソンショウ ト シンダン サレタ イチレイ

Congenital antithrombin III (AT III) deficiency is an inherited autosomal dominant disorder. Patients often suffer from recurrent venous thromboses that are triggered by several occasions (operation, gestation, trauma, oral contraceptive drug etc.). Moreover, 60% of them are said to be associated with pulmonary embolism. The patient of this report is 27-year-old pregnant woman in the first trimester. She felt pain in the back of her head and left auricle and presented with dyslexia and aphasia in late of March, 20XX. Getting CT brain scan, MRI brain scan, and blood sampling at the nearby hospital, she was suspected of having thrombosis of left sigmoid and transverse sinus due to AT III deficiency. Because she wanted to give birth to her first child without termination, she was referred to our hospital. We used heparin as the anticoagulant therapy because warfarin had the risk of teratogenesis. But in condition of low serum level of AT III activity, it didn’t work effectively. So we also did frequent complement of AT III. Strict anticoagulant therapy resulted in better outcome for both the patient and her baby without fatal venous thromboses or fetal complications