import json

data = {
 "topic": "Hypoplastic Left Heart Syndrome",
 "slug": "hypoplastic-left-heart-syndrome",
 "category_id": 15007,
 "summary": "Hypoplastic left heart syndrome is the most severe congenital heart lesion, in which underdevelopment of the entire left heart forces the right ventricle to support both circulations via a ductus-dependent systemic flow that decompensates rapidly when the ductus closes.",
 "written_by": "claude-sonnet",
 "references": [
  {"title": "Kliegman R. Nelson Textbook of Pediatrics 2-Volume Set 22ed 2024", "author": None, "pages": [2840, 2841, 2847]},
  {"title": "MedStudy Pediatrics Core 11th Edition 2024-2025", "author": None, "pages": [514, 530]},
  {"title": "Cover", "author": "Vitalsource Download", "pages": [7002, 7160, 7428]},
  {"title": "Illustrated Textbook of Paediatrics (Tom Lissauer, Will Carroll)", "author": "Lissauer, Tom,Carroll, Will", "pages": [350, 351]},
  {"title": "Diagnostic Imaging: Pediatrics", "author": "A. Carlson Merrow Jr. MD", "pages": [291, 307]},
  {"title": "Common Cardiac Issues in Pediatrics", "author": "Jonathan N. Johnson,Deepak M. Kamat", "pages": [225]}
 ],
 "short": [
  {"title": "In short", "content": (
   "- Hypoplastic left heart syndrome (HLHS) is marked underdevelopment of the entire left side of the heart: aortic and/or mitral valve stenosis/atresia, a hypoplastic left ventricle, and a diminutive ascending aorta\n"
   "- Three anatomic subtypes: mitral atresia with aortic atresia (MA-AA), mitral stenosis with aortic atresia (MS-AA), and mitral stenosis with aortic stenosis (MS-AS)\n"
   "- Accounts for roughly 25% of all cardiac deaths in the first year of life\n"
   "- The right ventricle is dilated and hypertrophied and supports both pulmonary and systemic circulation, the latter through a patent ductus arteriosus (PDA) - the lesion is ductus-dependent for systemic flow\n"
   "- Almost invariably associated with coarctation of the aorta and a diminutive ascending aorta (often under 5 mm)\n"
   "- Most infants appear normal at birth because the PDA is still open; they become acutely ill with poor perfusion, lactic acidosis, heart failure, and/or cardiogenic shock as the ductus closes\n"
   "- Presentation includes cyanosis with a grayish color, weak/poor pulses, and a hyperdynamic right ventricular impulse\n"
   "- Diagnostic echo findings: small left ventricular chamber whose apex does not form the cardiac apex, and a left-to-right bowing atrial septum indicating a pressure-restrictive interatrial communication\n"
   "- A highly restrictive atrial septum can be managed with balloon atrial septostomy, septal balloon dilation, stenting, or rarely blade septostomy\n"
   "- Ventilator strategy to balance pulmonary and systemic flow: keep PCO2 around 45-50 mmHg and avoid supplemental oxygen (a pulmonary vasodilator) when systemic arterial saturation is 70-80%"
  )}
 ],
 "long": [
  {"title": "Definition", "content": (
   "Hypoplastic left heart syndrome (HLHS) describes a group of related anomalies characterized by varying degrees of underdevelopment of the left side of the heart: stenosis or atresia of the aortic and/or mitral valves, hypoplasia of the left ventricular cavity, and marked hypoplasia of the ascending aorta. Three anatomic categories are recognized based on the valve findings: mitral atresia with aortic atresia (MA-AA), mitral stenosis with aortic atresia (MS-AA), and mitral stenosis with aortic stenosis (MS-AS). The left ventricle itself may be only moderately hypoplastic, very small and nonfunctional, or completely atretic; its cavity can be slit-like, miniaturized, or small and thick-walled with endocardial fibroelastosis. Coarctation of the aorta almost invariably accompanies HLHS."
  )},
  {"title": "Epidemiology", "content": (
   "HLHS is the most severe congenital heart lesion and accounts for nearly 25% of all cardiac deaths in the first year of life, based on data from the New England Regional Infant Cardiac Registry."
  )},
  {"title": "Pathophysiology", "content": (
   "Because the left heart cannot support systemic output, the right ventricle is dilated and hypertrophied and takes over both circulations: it ejects into the pulmonary artery to supply the lungs, and systemic perfusion is maintained retrograde through a patent ductus arteriosus (PDA) into the hypoplastic ascending aorta and coronary/cerebral circulation. Pulmonary venous return enters the left atrium and crosses an interatrial communication to reach the right heart. This arrangement makes systemic perfusion entirely ductus-dependent. In patients with mitral stenosis and aortic atresia (MS-AA), anomalous connections may exist between the left ventricle and the coronary arteries, which some reports link to increased postoperative risk. Most infants are hemodynamically stable at birth while the ductus remains patent, but as it closes (typically in the first days of life) systemic blood flow collapses, producing poor perfusion, lactic acidosis, acute heart failure, and/or cardiogenic shock."
  )},
  {"title": "Clinical features", "content": (
   "Affected infants are usually born appearing well, unless the diagnosis was already made on antenatal ultrasound. As the ductus arteriosus narrows and closes, infants develop signs of poor systemic perfusion: cyanosis with a grayish skin color, weak or poor pulses that progressively worsen, and a hyperdynamic right ventricular precordial impulse. Without intervention, this progresses to cardiogenic shock. Chest radiograph findings include cardiomegaly, an enlarged right atrium, and pulmonary venous congestion with interstitial edema, reflecting increased pulmonary blood flow and left-to-right shunting across the atrial septal defect."
  )},
  {"title": "Diagnostics", "content": (
   "Echocardiography is diagnostic, showing a small left ventricular chamber whose apex fails to form the cardiac apex, hypoplasia or atresia of the aortic and/or mitral valves, and a diminutive ascending aorta (often less than 5 mm). The atrial septum characteristically bows from left to right, indicating a pressure-restrictive interatrial communication. Cross-sectional imaging (CT/MRI) can assess the patency of surgical shunts (e.g., a Blalock-Taussig shunt or Glenn shunt) after staged palliation, and MRI is used for functional assessment of the single ventricle to determine suitability for a Fontan operation. Fetal echocardiography can identify a highly restrictive intra-atrial septum before birth (with findings such as bowing of the atrial septum and altered pulmonary venous Doppler flow patterns), which may prompt in-utero intervention such as atrial septal stenting."
  )},
  {"title": "Treatment", "content": (
   "Initial stabilization in the neonatal period focuses on maintaining ductal patency (to preserve systemic flow) and balancing the pulmonary and systemic circulations, since an infant with HLHS is at risk of pulmonary overcirculation stealing flow from the systemic circuit. Ventilator management aims to keep PCO2 in the 45-50 mmHg range and to avoid supplemental oxygen (which acts as a pulmonary vasodilator and can worsen the imbalance) when systemic arterial oxygen saturation is in the 70-80% range - this helps prevent systemic hypoperfusion. If the atrial septum is restrictive, mechanical intervention may be required: Rashkind balloon atrial septostomy, septal balloon dilation, or, rarely, blade septostomy. Definitive management is staged surgical palliation of the single-ventricle circulation (culminating in a Fontan-type circulation), or in more severe cases with echocardiographically-assessed severity, catheter-based stenting."
  )},
  {"title": "Complications", "content": (
   "HLHS is a ductus-dependent lesion, so failure to recognize the diagnosis before ductal closure leads to profound cardiogenic shock and death. Even with treatment, staged single-ventricle palliation carries ongoing risks related to the single functioning ventricle and the surgical shunts and connections used at each stage. In MS-AA anatomy specifically, anomalous coronary-to-ventricular connections have been associated with increased postoperative risk."
  )}
 ],
 "clinical": [
  {"title": "Management at the bedside", "content": (
   "Suspect HLHS in any neonate who develops sudden poor perfusion, cyanosis with a grayish hue, weakening pulses, or cardiogenic shock in the first days of life, particularly if antenatal ultrasound was not performed or was normal - the clinical deterioration typically coincides with ductal closure. Confirm with echocardiography looking for a small, apex-forming-failing left ventricle, aortic/mitral valve hypoplasia or atresia, a diminutive ascending aorta, and a left-to-right bowing, pressure-restrictive atrial septum.\n\nOnce suspected or confirmed, initial stabilization centers on maintaining ductal patency and balancing the pulmonary and systemic circuits. Titrate ventilation to keep PCO2 around 45-50 mmHg, and avoid supplemental oxygen when systemic saturation is running 70-80%, since oxygen acts as a pulmonary vasodilator and can divert flow away from the systemic circulation, worsening perfusion. If the atrial septum is restrictive on echo, escalate to catheter-based intervention - Rashkind balloon atrial septostomy, septal balloon dilation, or rarely blade septostomy - to relieve pulmonary venous congestion. Definitive care requires transfer to a center capable of staged single-ventricle surgical palliation."
  )}
 ]
}

with open("/tmp/hlhs.article.json", "w") as f:
    json.dump(data, f, indent=1)
print("written")