import json

data = {
 "topic": "Bicuspid Aortic Valve",
 "slug": "bicuspid-aortic-valve",
 "category_id": 15569,
 "summary": "Bicuspid aortic valve is the most common congenital heart defect, affecting 1-2% of the population, often asymptomatic for decades but requiring lifelong echocardiographic surveillance for progressive stenosis, regurgitation, and aortic dilation/dissection.",
 "written_by": "claude-sonnet",
 "references": [
  {"title": "Cover", "author": "Vitalsource Download", "pages": [7113]},
  {"title": "Nelson Pediatrics Board Review - Certification And Recertification-", "author": None, "pages": [46]},
  {"title": "Common Cardiac Issues in Pediatrics", "author": "Jonathan N. Johnson,Deepak M. Kamat", "pages": [13, 30, 301, 306, 310, 752]},
  {"title": "Netters Pediatrics (Florin Т., Ludwig St.)", "author": None, "pages": [286]},
  {"title": "CURRENT Diagnosis and Treatment Pediatrics, Twenty-Fourth Edition", "author": "Hay, William W., Levin, Myron J., Deterding, Robin R., Abzug, Mark J.", "pages": [619]},
  {"title": "MedStudy Pediatrics Core 11th Edition 2024-2025", "author": None, "pages": [517]},
  {"title": "Kliegman R. Nelson Textbook of Pediatrics 2-Volume Set 22ed 2024", "author": None, "pages": [2814]}
 ],
 "short": [
  {"title": "In short", "content": (
   "- Bicuspid aortic valve (BAV) is the most common congenital heart defect, present in 1-2% of the general population\n"
   "- Defined by fusion of the commissure between two of the normally three aortic valve cusps; anatomy is variable, ranging from truly only 2 cusps to partial fusion of an otherwise tricuspid valve\n"
   "- There is a genetic component - BAV and other left-sided obstructive lesions (coarctation, hypoplastic left heart syndrome) tend to cluster in families\n"
   "- BAV is found in about 50% of patients with coarctation of the aorta, but more often occurs as an isolated anomaly\n"
   "- A systolic ejection click or thrill in the suprasternal notch suggests BAV and is present in about 70% of cases; a short systolic murmur is often heard along the left sternal border (3rd-4th intercostal space), sometimes with an additional grade 1-2/6 systolic ejection murmur/click at the right upper sternal border - though many cases are not clinically apparent at all\n"
   "- Diagnosis is best made by 2-dimensional echocardiography\n"
   "- Natural history: valvar calcification and sclerosis may begin as early as the teenage years; the likelihood of eventually developing calcific aortic stenosis (or, less often, regurgitation) is approximately 75%\n"
   "- BAV predisposes to progressive aortic dilation and dissection (grouped with Marfan syndrome, Loeys-Dietz syndrome, Turner syndrome, and type IV Ehlers-Danlos syndrome as at-risk conditions); poststenotic aortic dilation is common, and the aortic wall itself is often abnormal (sometimes showing cystic medial necrosis), though ascending aortic dissection is very rare under age 40\n"
   "- Antibiotic prophylaxis against infective endocarditis is no longer officially recommended for an isolated bicuspid aortic valve, even when associated with simple coarctation of the aorta\n"
   "- Because BAV can remain silent for decades before manifesting valve dysfunction or aortic complications, affected patients require periodic lifelong screening even if asymptomatic at diagnosis"
  )}
 ],
 "long": [
  {"title": "Definition", "content": (
   "The aortic valve normally has three thin cusps (tricuspid/trileaflet) anchored to the valve annulus, just distal to which the aorta bulges to form the aortic root before narrowing again at the sinotubular junction. A bicuspid aortic valve (BAV) is defined by fusion of the commissure between two of the three cusps, and is the most common congenital aortic valve anomaly and the most common congenital heart defect overall. The anatomy of BAV varies, ranging from the presence of only two true cusps to partial fusion of an otherwise tricuspid valve. BAV produces an asymmetric valve orifice; the valve may not open fully during systole, but this need not cause any obstruction to left ventricular ejection."
  )},
  {"title": "Epidemiology", "content": (
   "BAV is present in 1-2% of the general population, making it the most common congenital heart defect. It is found in approximately 50% of patients with coarctation of the aorta, though it more often occurs as an isolated anomaly without other structural heart disease. There is a genetic component to BAV: it and other left-sided obstructive lesions - including hypoplastic left heart syndrome - tend to cluster within families."
  )},
  {"title": "Clinical features", "content": (
   "BAV may be asymptomatic for many years, and in many patients it is not clinically apparent at all on routine examination. When findings are present, a systolic ejection click or thrill in the suprasternal notch is characteristic, reported in about 70% of cases, and a short systolic murmur is often heard along the left sternal border at the third and fourth intercostal spaces, well transmitted to the left infrascapular area and occasionally to the neck. Some patients instead have a milder grade 1-2/6 systolic ejection murmur and click at the right upper sternal border. If aortic stenosis develops, the typical murmur can be heard at the upper right and mid-left sternal borders."
  )},
  {"title": "Diagnostics", "content": (
   "Two-dimensional echocardiography is the diagnostic modality of choice for identifying BAV and characterizing the valve anatomy, associated stenosis or regurgitation, and any coexisting aortic dilation."
  )},
  {"title": "Pathophysiology", "content": (
   "Not all bicuspid valves are stenotic or regurgitant at diagnosis, but valvar calcification and sclerosis can begin as early as the teenage years, and the leaflets tend to develop progressive dysfunction over months to decades. In middle-aged adults, calcific aortic stenosis is usually found to arise from a congenitally bicuspid valve, and the overall likelihood of a BAV patient eventually developing calcific aortic stenosis, or less commonly aortic regurgitation, is approximately 75%. BAV is also associated with abnormalities of the aortic wall itself, sometimes showing cystic medial necrosis, which predisposes to poststenotic dilation of the aorta and, rarely, dissection of the ascending aorta (very uncommon before age 40). Because of this aortopathy, BAV is grouped alongside Marfan syndrome, Loeys-Dietz syndrome, Turner syndrome, and type IV Ehlers-Danlos syndrome as conditions carrying increased risk for progressive aortic dilation and dissection, independent of the valve's own function."
  )},
  {"title": "Treatment", "content": (
   "Because BAV can be silent for years while progressing toward significant valve dysfunction or aortic dilation, ongoing periodic clinical and echocardiographic surveillance throughout life is the cornerstone of management, even in asymptomatic patients. Antibiotic prophylaxis against infective endocarditis is no longer officially recommended for an isolated bicuspid aortic valve, including when it accompanies simple, uncomplicated coarctation of the aorta. Management of established aortic stenosis or regurgitation, or of significant aortic root/ascending aortic dilation, follows standard valve and aortopathy management pathways once these complications develop, guided by serial imaging."
  )},
  {"title": "Complications", "content": (
   "The major long-term complications of BAV are progressive aortic valve dysfunction (calcific aortic stenosis in the majority of eventually symptomatic patients, or less commonly regurgitation), poststenotic and aortic root/ascending aortic dilation, and, rarely, aortic dissection (essentially never before age 40). Occasionally, a bicuspid aortic valve becomes the site of infective endocarditis, though as noted, prophylaxis is no longer routinely recommended for this indication alone."
  )}
 ],
 "clinical": [
  {"title": "Follow-up and surveillance at the bedside", "content": (
   "When BAV is identified - whether incidentally on exam (systolic ejection click, suprasternal thrill, or a soft systolic murmur) or on echocardiography performed for another reason such as coarctation screening - confirm and characterize it with 2D echocardiography, since this is the definitive diagnostic tool. Establish a plan for lifelong periodic re-evaluation even in an asymptomatic child or adolescent, because valvar calcification/sclerosis can begin as early as the teenage years and roughly three-quarters of BAV patients eventually develop clinically significant stenosis or regurgitation over their lifetime.\n\nAt each surveillance echocardiogram, assess not only valve gradient/function but also the aortic root and ascending aorta, since BAV carries an intrinsic aortopathy (sometimes cystic medial necrosis) independent of valve function, predisposing to progressive dilation and, rarely, dissection. Do not routinely prescribe antibiotic endocarditis prophylaxis for an isolated BAV, even with associated simple coarctation - current guidance does not support it. If BAV is found in a child, screen first-degree relatives given the recognized familial clustering of BAV and other left-sided obstructive lesions, and counsel the family that the condition, while common and often benign for years, requires committed long-term follow-up rather than a one-time reassurance."
  )}
 ]
}

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