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2025 ICD-10-CM code Q23.4

Hypoplastic left heart syndrome (HLHS) is a critical congenital heart defect where the left side of the heart is underdeveloped.

Coding should accurately reflect the severity and specific features of the HLHS.Appropriate use of ICD-10 codes requires a thorough understanding of the clinical picture.

Not applicable to ICD-10 codes.

Medical necessity for HLHS diagnosis and treatment is based on the presence of clinical symptoms, echocardiographic findings consistent with HLHS, and the need for interventions to address the significant cardiovascular compromise caused by this life-threatening condition.

Diagnosis and management of HLHS are typically the responsibility of pediatric cardiologists and cardiac surgeons.Other specialists, such as neonatologists and intensivists may also be involved in the care of these patients.

IMPORTANT:ICD-11 code LA89.3 is a potential alternate code.

In simple words: Hypoplastic left heart syndrome (HLHS) is a serious heart problem a baby is born with.The left side of the baby's heart doesn't develop properly, making it hard for the heart to pump enough blood to the body.Babies with HLHS need special medical care and often require surgery.

Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect characterized by underdevelopment of the left side of the heart, including the left ventricle, ascending aorta, aortic arch, and mitral and/or aortic valve. This results in the left heart's inability to provide sufficient systemic blood flow.The condition's severity varies, with subtypes distinguished by the status of the mitral and aortic valves.The most severe subtype involves atresia of both valves, a severely underdeveloped or absent left ventricle, and a diminutive aortic arch. Other subtypes exhibit variable ventricular hypoplasia with a discernible left ventricular cavity and varying degrees of mitral and/or aortic stenosis.Diagnosis is typically made using echocardiography, which reveals hypoplasia or atresia of the left-sided structures. Newborns with HLHS may initially appear healthy but become dependent on the patent ductus arteriosus (PDA) for systemic circulation. PDA closure leads to decreased systemic perfusion, resulting in hypoxemia, acidosis, and cardiogenic shock.Treatment usually involves complex cardiac surgery.

Example 1: A newborn infant presents with cyanosis and respiratory distress.Echocardiography reveals hypoplasia of the left ventricle and aortic atresia, consistent with HLHS. The infant is transferred to a pediatric cardiac center for surgical intervention., A pregnant woman undergoes fetal echocardiography at 20 weeks gestation which reveals HLHS. The parents are counseled regarding the condition and its management, including options for post-natal surgical intervention., A 3-month-old infant with a history of HLHS surgery is admitted to the hospital with signs of congestive heart failure.Further evaluation and treatment are required to manage the ongoing cardiac complications.

Detailed prenatal history (if available), physical examination findings, echocardiogram reports, cardiac catheterization reports (if performed), surgical reports (if applicable), and laboratory results (e.g., blood gas analysis).Progress notes should document the clinical course, treatment interventions, and response to treatment.

** This code should be used to report the diagnosis of Hypoplastic Left Heart Syndrome.It is not intended for use in maternal records.Further codes may be necessary to capture additional congenital heart defects or complications.

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