{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T15:05:08Z","timestamp":1784905508706,"version":"3.55.0"},"reference-count":42,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T00:00:00Z","timestamp":1784851200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Background\/Objectives: Isolated Left Ventricular Apical Hypoplasia (ILVAH) is a rare congenital anomaly characterized by a truncated, spherical left ventricle (LV) with fibro-fatty apical replacement. Historically considered a benign condition of asymptomatic adults, its hemodynamic behavior under physiological stress remains poorly characterized. We present two distinct cases and a comprehensive literature review (n = 59) to redefine the clinical spectrum of ILVAH. Methods: To contextualize our findings, a comprehensive review of the literature was performed up to February 2026. We searched the PubMed\/MEDLINE database using the terms \u201cIsolated Left Ventricular Apical Hypoplasia\u201d, \u201cILVAH\u201d, \u201ctruncated left ventricle\u201d, and \u201cleft ventricular apical hypoplasia\u201d. The literature search and study selection were conducted in accordance with the PRISMA guidelines. Case presentations: Case 1 describes a male infant with ILVAH and muscular ventricular septal defects who unexpectedly developed transient systolic heart failure at one month of life, requiring prompt medical therapy (ACE inhibitors and diuretics) for functional recovery. Case 2 describes a 33-year-old woman with a known diagnosis of ILVAH. Serial echocardiography during her first pregnancy revealed progressive left atrial dilation and the onset of mild post-capillary pulmonary hypertension. Immediately following an elective cesarean section at 37 weeks, she experienced acute heart failure. She was successfully managed with pre-emptive volume offloading. Conclusions: ILVAH is not a universally benign anomaly. The morphologically deficient and rigid ventricle is vulnerable to afterload shifts in infancy and rapid volume expansion in adulthood. A review of all previously reported pregnancies in ILVAH reveals a consistent pattern of severe, unrecognized postpartum pulmonary edema. Proactive, multidisciplinary management is suggested to prevent clinical decompensation. Our findings suggest that ILVAH may act as a stress-sensitive restrictive congenital left ventricle disease rather than a universally benign anatomical variant, a hypothesis that warrants further investigation.<\/jats:p>","DOI":"10.3390\/children13080983","type":"journal-article","created":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T13:53:26Z","timestamp":1784901206000},"page":"983","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Isolated Left Ventricular Apical Hypoplasia, from Transient Neonatal Dysfunction to Maternal Hemodynamic Stress: A Comprehensive Review with Illustrative Cases"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-8250-6653","authenticated-orcid":false,"given":"Mattia","family":"Pasquinucci","sequence":"first","affiliation":[{"name":"Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"},{"name":"Department of Pediatrics, AULSS 7 Pedemontana\u2014San Bassiano Hospital, 36061 Bassano del Grappa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4970-4562","authenticated-orcid":false,"given":"Martina","family":"Avesani","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Department of Woman\u2019s and Child\u2019s Health, Padua University Hospital, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"La Rosa","sequence":"additional","affiliation":[{"name":"Department of Women\u2019s and Children\u2019s Health, Padua University Hospital, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2493-3708","authenticated-orcid":false,"given":"Maria Elena","family":"Derchi","sequence":"additional","affiliation":[{"name":"Cardiology Unit, Department of Surgical Sciences, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-2893-3868","authenticated-orcid":false,"given":"Davide","family":"Meneghesso","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, AULSS 7 Pedemontana\u2014San Bassiano Hospital, 36061 Bassano del Grappa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Buffi","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology Unit, Department of Critical Care and Perinatal Medicine, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7793-714X","authenticated-orcid":false,"given":"Federico","family":"Prefumo","sequence":"additional","affiliation":[{"name":"Obstetrics and Gynecology Unit, Department of Critical Care and Perinatal Medicine, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Tralli","sequence":"additional","affiliation":[{"name":"Department of Obstetrics, Gynecology and Fetal Medicine, AULSS 8 Berica\u2014San Bortolo Hospital, 36100 Vicenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michela","family":"Marchesini","sequence":"additional","affiliation":[{"name":"Department of Obstetrics, Gynecology and Fetal Medicine, AULSS 8 Berica\u2014San Bortolo Hospital, 36100 Vicenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Nocerino","sequence":"additional","affiliation":[{"name":"Department of Obstetrics, Gynecology and Fetal Medicine, AULSS 8 Berica\u2014San Bortolo Hospital, 36100 Vicenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Grison","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Neonatal Intensive Care, AULSS 8 Berica\u2014San Bortolo Hospital, 36100 Vicenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Santagati","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Neonatal Intensive Care, AULSS 8 Berica\u2014San Bortolo Hospital, 36100 Vicenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Bordin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Neonatal Intensive Care, AULSS 8 Berica\u2014San Bortolo Hospital, 36100 Vicenza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriele","family":"De Tonetti","sequence":"additional","affiliation":[{"name":"Obstetric Anesthesia, Department of Critical Care and Perinatal Medicine, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena Sofia","family":"Milandri","sequence":"additional","affiliation":[{"name":"Unit of Obstetrics and Gynecology, Santa Maria Delle Croci Hospital, AUSL Romagna, 48121 Ravenna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0305-0113","authenticated-orcid":false,"given":"Giovanni","family":"Di Salvo","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Department of Woman\u2019s and Child\u2019s Health, Padua University Hospital, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5069-7067","authenticated-orcid":false,"given":"Gianluca","family":"Trocchio","sequence":"additional","affiliation":[{"name":"Cardiology Unit, Department of Surgical Sciences, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,7,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"552","DOI":"10.1136\/hrt.2003.010637","article-title":"Isolated left ventricular apical hypoplasia: A new congenital anomaly described with cardiac tomography","volume":"90","author":"Srichai","year":"2004","journal-title":"Heart"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"e15793","DOI":"10.1111\/echo.15793","article-title":"Multimodality imaging for the diagnosis of left ventricular apical hypoplasia: A case presentation and review of the literature","volume":"41","author":"Liao","year":"2024","journal-title":"Echocardiography"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"5494","DOI":"10.12998\/wjcc.v11.i23.5494","article-title":"Isolated left ventricular apical hypoplasia: Systematic review and analysis of the 37 cases reported so 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Imaging"}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/13\/8\/983\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T14:15:20Z","timestamp":1784902520000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/13\/8\/983"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,24]]},"references-count":42,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2026,8]]}},"alternative-id":["children13080983"],"URL":"https:\/\/doi.org\/10.3390\/children13080983","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7,24]]}}}