{
    "abstract": "A partial atrioventricular septal defect (AVSD) with a hypoplastic left ventricle and common atrium is a rare combination of cardiac anomalies that can be associated with Ellis-van Creveld (EVC) syndrome.\nA female neonate with EVC syndrome was diagnosed with an unbalanced AVSD and hypoplastic left ventricle. Pulmonary artery banding and ductus ligation were performed at 23 days after birth. The postoperative course was complicated by moderate atrioventricular valve (AVV) regurgitation and low cardiac output. On postoperative Day 6, emergent extracorporeal membrane oxygenation (ECMO) was performed to treat acute circulatory failure. Under suspicion of systemic ventricular outflow tract obstruction, the Damus-Kaye-Stansel procedure with a systemic-to-pulmonary artery shunt and AVV plasty were performed. Intraoperatively, no ventricular septal defect was found. The right and left AVV orifices were found to be separated. Postoperatively, the patient could not be weaned from cardiopulmonary bypass and continued to receive ECMO support. Eight days postoperatively, a right ventricle-to-pulmonary artery shunt and division of the systemic-to-pulmonary artery shunt were performed to increase the pulmonary blood flow. On postoperative Day 5, the ECMO was successfully removed under continuous infusion of adrenalin, but the patient died of severe renal failure 4 days later. The parents consented to autopsy. The heart was permanently preserved by perfusion-distention fixation and wax infiltration.\nWe reported a rare combination of cardiac defects of common atrium, partial AVSD, and hypoplastic left ventricle associated with EVC syndrome. Accurately diagnosing the presence of ventricular septal defect is essential part in determining surgical treatment strategy.",
    "authors": [
        {
            "affiliation": "Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Suita, Osaka 565-0871, Japan.",
            "firstname": "Takashi",
            "initials": "T",
            "lastname": "Kido"
        },
        {
            "affiliation": "Department of Thoracic and Cardiovascular Surgery, Mie University Graduate School of Medicine, Tsu, Mie 514-8507, Japan.",
            "firstname": "Shuhei",
            "initials": "S",
            "lastname": "Toba"
        },
        {
            "affiliation": "Department of Thoracic and Cardiovascular Surgery, Mie University Graduate School of Medicine, Tsu, Mie 514-8507, Japan.",
            "firstname": "Takato",
            "initials": "T",
            "lastname": "Yamasaki"
        },
        {
            "affiliation": "Department of Cardiovascular Surgery, Osaka Women's and Children's Hospital, Izumi, Osaka 594-1101, Japan.",
            "firstname": "Dai",
            "initials": "D",
            "lastname": "Asada"
        },
        {
            "affiliation": "Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka 594-1101, Japan.",
            "firstname": "Sanae",
            "initials": "S",
            "lastname": "Tsumura"
        }
    ],
    "conclusions": null,
    "copyrights": "\u00a9 The Author(s) 2025. Published by Oxford University Press on behalf of the European Society of Cardiology.",
    "doi": "10.1093/ehjcr/ytaf005",
    "journal": "European heart journal. Case reports",
    "keywords": [
        "Case report",
        "Common atrium",
        "Ellis\u2013van Creveld syndrome",
        "Partial atrioventricular septal defect",
        "Perfusion\u2013distention fixation"
    ],
    "methods": null,
    "publication_date": "2025-01-28",
    "pubmed_id": "39872675\n24168757\n34973431\n10700184\n21070693\n21533779",
    "results": null,
    "title": "A case report of a rare cardiac anomaly associated with Ellis-van Creveld syndrome: common atrium, partial atrioventricular septal defect, and hypoplastic left ventricle.",
    "xml": "<Element 'PubmedArticle' at 0x7885035914e0>"
}