Hossin Huda, Sleiay Mouhammed, Kouran Joudi, Alsuliman Raghad Mahmoud, Kojok Mhd Anas, Alkhateb Nour Mohamad Najeb, Ghanem Suha Giselle, Mansour Marah, Alsuliman Tamim, Takkem Saleh
Faculty of Medicine.
Faculty of Medicine, Damascus University.
Ann Med Surg (Lond). 2024 Apr 24;86(7):4146-4151. doi: 10.1097/MS9.0000000000002041. eCollection 2024 Jul.
Univentricular heart disease is a relatively rare condition that affects infants, with a prevalence ranging from 0.05 to 0.1 per 1000 live births. It is characterized by an abnormality in the structure of the heart, specifically the presence of only one main pumping chamber (ventricle) instead of the usual two.
In this particular case, a newborn male was diagnosed with double-inlet left ventricle (DILV), a specific form of univentricular heart disease. Following his birth, he exhibited symptoms of central cyanosis (a bluish tint to the skin due to poor oxygenation) and difficulties with breastfeeding. Clinical evaluation, along with a heart ultrasound, confirmed the need for palliative surgery. At the age of 6 months, the patient is scheduled to undergo the Glenn procedure, a surgical intervention that aims to redirect blood flow to the lungs and improve oxygenation.
Given the complexity of double-inlet single ventricle anomalies, there are multiple differential diagnoses that need to be considered for accurate diagnosis, including conditions such as tricuspid atresia, large ventricular septal defect and corrected transposition of the great arteries with ventricular septal defect.
Early intervention in the immediate postnatal period plays a crucial role in improving survival rates and reducing long-term complications. It is, therefore, essential to continue researching and refining treatment approaches.
单心室心脏病是一种相对罕见的影响婴儿的疾病,每1000例活产婴儿中的患病率为0.05至0.1。其特征是心脏结构异常,具体表现为只有一个主要的泵血腔(心室),而非正常的两个。
在这个特殊病例中,一名新生男婴被诊断为双入口左心室(DILV),这是单心室心脏病的一种特殊形式。出生后,他出现了中心性紫绀(因氧合不足导致皮肤呈蓝色)和母乳喂养困难的症状。临床评估以及心脏超声检查证实需要进行姑息性手术。在6个月大时,该患者计划接受格林手术,这是一种旨在重新引导血液流向肺部并改善氧合的手术干预。
鉴于双入口单心室异常的复杂性,为了准确诊断,需要考虑多种鉴别诊断,包括三尖瓣闭锁、大型室间隔缺损以及合并室间隔缺损的矫正型大动脉转位等情况。
出生后早期干预在提高生存率和减少长期并发症方面起着至关重要的作用。因此,继续研究和完善治疗方法至关重要。