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Background Congenital porto‐systemic shunt (CPSS) is a rare disease and can cause fatal complications. Accurate angiographic assessment is mandatory for proper treatment. Although technically difficult, we developed assessment techniques and assessed their accuracy. One technique came from evaluating patients with extrahepatic portal vein obstruction (EHPVO). Methods We conducted a single center retrospective study to evaluate the efficacy of angiographic diagnostic procedure for the assessment of CPSS and EHPVO, and its impact on patients’ subsequent interventions and clinical course. Eight patients with CPSS and two patients with EHPVO who underwent diagnostic angiography were included. Assessment of the intrahepatic portal vein was performed in all patients. The route of the shunt, and portal vein pressure under shunt occlusion, were also evaluated for patients with CPSS. Evaluation was first attempted with a balloon angiographic catheter (standard method). Three additional techniques were performed as needed: (i) direct wedge‐catheter injection without balloon inflation, (ii) use of occlusion balloon in two patients, and (iii) hybrid angiography with sheath placement directly into the superior mesenteric vein. Results The standard method was sufficient in four patients. On the other hand, all three techniques were required in two patients each. One lost contact during follow up, but all other patients underwent optimal intervention. There were no complications related to the angiographic procedure. Conclusions Use of direct wedge‐catheter injection without balloon inflation, occlusion balloon, and hybrid catheterization improved the diagnostic yield in patients with CPSS or EHPVO.

Mycophenolate mofetil (MMF) is used widely to treat lupus nephritis and is considered safer than other immunosuppressive drugs. Reports on severe complications related to MMF are sparse. We report a case of a patient with lupus nephritis in whom severe complications were possibly caused by MMF. The patient was a 17-year-old girl who received a diagnosis of lupus nephritis at the age of 14 years and had been taking steroid and immunosuppressive agents since then. One week after starting MMF 1 g/day instead of mizoribine owing to symptom relapse and serologic data deterioration, she presented with seizure, accompanied by leukopenia, thrombocytopenia, and renal failure. We discontinued MMF because she had extremely high serum mycophenolate acid concentration (88 µg/mL). A few weeks later, she recovered without any complications and was discharged. Although rare, clinicians should be aware that serum mycophenolate acid concentration may become extremely high in the setting of acute kidney injury. In such circumstances, they should perform serum concentration monitoring to avoid possible adverse events.

Background Hypoplastic coronary artery disease (HCAD) is an extremely rare disease associated with a risk of sudden cardiac death. It is rarely recognized in a live pediatric patient. Case summary We report a case of HCAD in a patient who first presented with vomiting and poor feeding, suggestive of acute heart failure due to cardiomyopathy or acute myocarditis in infancy. Hypertension and signs of ischemia became evident on electrocardiography and scintigraphy after his cardiac function fully recovered, and he was diagnosed with HCAD by angiography performed at the age of eight years. He has remained under close observation with anti-hypertensives, aspirin, and exercise restriction. Discussion Although HCAD is a rare disease, it may not only cause ischemia but may also result in heart failure and sudden cardiac death. It should be considered in any pediatric patient with heart failure. Mid-term follow-up visits might be necessary to detect signs of ischemia in pediatric patients presenting with features of heart failure.

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