Chiaki Iida

Successful Treatment of Protein-Losing Enteropathy and Plastic Bronchitis by Biphasic Cuirass Ventilation in a Patient with Failing Fontan Circulation

We present a 16-year-old male patient with hypoplastic left heart syndrome who developed protein-losing enteropathy (PLE) and plastic bronchitis (PB) after a Fontan operation. He received medical therapies, including albumin infusion, unfractionated heparin, and high-dose anti-aldosterone therapy but could not obtain clinical relief. Biphasic cuirass ventilation (BCV) led to expectoration of bronchial casts and prompt resolution of PB. Notably, clinical symptoms related to PLE were dramatically improved after starting BCV. A brief period of BCV increased stroke volume from 26±1.4 to 39±4.0 mL. This case suggests that BCV could be an effective treatment for PLE in patients with failing Fontan circulation.

Transcatheter Closure of a Huge Ductus Arteriosus in a Severely Ill Neonate

Hemodynamically significant patent ductus arteriosus (PDA) in preterm infants increases morbidity and mortality. Here we describe a 12-day-old neonate with a huge PDA who developed pulmonary hemorrhage following disseminated intravascular clotting and multiple organ failure. Medical treatment or surgical ligation could not be performed because of the patients poor condition. Transcatheter closure using a commercially available device (Amplatzer Vascular Plug II) successfully treated the huge PDA without major complications. The Amplatzer Vascular Plug II approach might become a new option for PDA closure in small infants, including those who are critically ill.

Unique levoatriocardinal veins in neonates with hypoplastic left heart syndrome and intact atrial septum

We had two cases of neonates with hypoplastic left heart syndrome and intact atrial septum who had unique levoatriocardinal veins. Contrast-enhanced CT and angiography revealed that previously unknown communicating vessels ran from the top of the left atrium and drained into the right atrium. We emphasise that transcatheter atrial septostomy should be performed not through these communicating vessels but using the trans-septal approach in neonates with hypoplastic left heart syndrome and an intact atrial septum.

Intravascular Ultrasound-Guided Cardiac Intervention in a Fontan Patient with Allergy to Contrast Media

Allergy to contrast media is hazardous in patients who need multiple cardiac catheterizations. We describe the case of a 16-year-old boy who presented with fatigue and cyanosis. He had undergone a Fontan operation 14 years previously, after which he developed an allergy to iodinated contrast media. Contrast echocardiograms by manual injection of microbubbles suggested the presence of a venovenous collateral vessel from the brachiocephalic vein draining into the pulmonary vein. We used intravascular ultrasound as the sole imaging method to identify the target vessel and to guide deployment of an Amplatzer Vascular Plug II. The patient experienced immediate improvement in his systemic saturation. Our experience shows that intravascular ultrasound may be a feasible alternative to standard imaging methods in patients with congenital heart disease who need cardiac interventional procedures and are allergic to contrast media.

Anaplastic Lymphoma Kinase-Positive Anaplastic Large Cell Lymphoma with Cardiac Metastasis and Arterial Tumor Embolisms during First-Course Chemotherapy.

We described an 11-year-old boy suffering from pediatric anaplastic lymphoma kinase-positive anaplastic large cell lymphoma with heart metastasis at diagnosis and arterial tumor embolisms during chemotherapy. Both the heart metastasis and pericardial effusion showed improvement with prednisolone, but numbness and pallor sequentially developed in his lower extremities during the first course of chemotherapy. Contrast-enhanced imaging revealed occlusion of the right anterior tibial artery and left popliteal artery. These symptoms were spontaneously remitted due to the compensation of other arteries. Arterial tumor embolism is a rare but possible complication when a lymphoma shows intracardiac infiltration.