Siddiqi Ahmed Kamal, Maniya Muhammad Talha, Ali Kumail Mustafa, Saqib Scheryar, Abdou Mahmoud H, Garcia Mariana, Quintana Raymundo A, Amin Sagar, Dabbagh Mohammed Ferras, Naeem Muhammad, De Cecco Carlo N
Division of Cardiothoracic Imaging, Department of Radiology and Imaging Sciences, Emory University, Clifton Road, NE, Atlanta, GA, 136430322, USA.
Department of Medicine, Ziauddin Medical University, Karachi, Pakistan.
Pediatr Cardiol. 2025 Aug 11. doi: 10.1007/s00246-025-03986-3.
Hypoplastic left heart syndrome (HLHS) is a complex congenital heart defect, characterized by hypoplasia of the left ventricle, the ascending aorta, and the aortic arch often with accompanying aortic and mitral valvular anomalies. If left untreated, HLHS is uniformly fatal in infancy, yet mortality trends remain unexplored. We aim to assess trends in HLHS-related mortality among the U.S. pediatric population. Death certificate data was analyzed from the CDC WONDER database for HLHS-related mortality from 1999 to 2020. Data were stratified by year, age, sex, race, urbanization status, state and census region. Age adjusted mortality rates (AAMR) and crude mortality rates (CMR) per 1,000,000 persons and annual percent changes (APC), were calculated. Between 1999 and 2020, 7053 HLHS-related deaths occurred among the pediatric population aged < 15 years. The overall AAMRs declined steadily from 1999 to 2020 (APC: - 2.54). Males consistently had higher AAMRs than females. Non-Hispanic (NH) Blacks had the highest AAMR in 2020 (4.96), followed by Hispanic/Latinos (3.78) and NH Whites (3.47). AAMRs varied between different regions (overall AAMR Northeast (3.95), Midwest (5.70), South (5.60), and West (5.09)). Urban areas (5.07) had lower HLHS-related AAMR than rural areas (5.93). States in the top 90th percentile of HLHS-related AAMRs (West Virginia, Utah, Iowa, Arkansas, and Maine) had approximately double the AAMRs than those in the bottom-10th percentile. The majority deaths occurred in infants < 1-year (CMR: 71.09), with mortality declining steeply thereafter and minimal CMRs (≤ 0.32) in children aged 9 to 14 years. HLHS-related mortality has declined steadily from 1999 to 2020. The highest AAMRs were observed in Black males and among children living in rural areas. Targeted strategies and public health initiatives are needed to further reduce the burden of HLHS-related mortality among the high-risk groups.
左心发育不全综合征（HLHS）是一种复杂的先天性心脏缺陷，其特征为左心室、升主动脉和主动脉弓发育不全，常伴有主动脉瓣和二尖瓣异常。若不进行治疗，HLHS在婴儿期会无一例外地导致死亡，但死亡率趋势仍未得到研究。我们旨在评估美国儿科人群中与HLHS相关的死亡率趋势。从疾病控制与预防中心（CDC）的WONDER数据库中分析了1999年至2020年与HLHS相关的死亡证明数据。数据按年份、年龄、性别、种族、城市化状况、州和人口普查区域进行分层。计算了每100万人的年龄调整死亡率（AAMR）、粗死亡率（CMR）和年度百分比变化（APC）。1999年至2020年期间，15岁以下儿科人群中有7053例与HLHS相关的死亡。从1999年到2020年，总体AAMR稳步下降（APC：-2.54）。男性的AAMR一直高于女性。2020年，非西班牙裔（NH）黑人的AAMR最高（4.96），其次是西班牙裔/拉丁裔（3.78）和NH白人（3.47）。不同地区的AAMR有所不同（总体AAMR东北部为3.95，中西部为5.70，南部为5.60，西部为5.09）。城市地区（5.07）与HLHS相关的AAMR低于农村地区（5.93）。HLHS相关AAMR处于第90百分位的州（西弗吉尼亚州、犹他州、爱荷华州、阿肯色州和缅因州）的AAMR约为处于第10百分位州的两倍。大多数死亡发生在1岁以下婴儿（CMR：71.09），此后死亡率急剧下降，9至14岁儿童的CMR最低（≤0.32）。1999年至2020年期间，与HLHS相关的死亡率稳步下降。黑人男性和农村地区儿童的AAMR最高。需要采取针对性策略和公共卫生举措，以进一步减轻高危人群中与HLHS相关的死亡负担。
