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Congenital Absence of Ductus Arteriosus

Absent Ductus Arteriosus


Presentation

  • Aortic valve stenosis was present in 3/5 in A but in 0/14 in B. ADA may be suspected clinically in cyanotic NB with reduced pulmonary flow if there is no response to PGE infusion and/or if aortic valve stenosis is also present.[nature.com]
  • If VSD is present it allows the baby to survive as the lung gets perfused by major or minor aortopulmonary collaterals. These collaterals can range between extensive and sparse.[drsvenkatesan.com]
  • Abstract Clinical, haemodynamic, angiocardiographic, and necropsy findings are reported in a newborn infant who presented with typical findings of the severe form of the syndrome of absent pulmonary valve.[heart.bmj.com]
  • Chest77:106–107 [5] David W, Reading, MS, and Umesh Oza (2012) Unilateral absence of a pulmonary artery: a rare disorder with variable presentation.[eurorad.org]
  • Clinical description TAR syndrome presents with bilateral absence of the radius with present thumbs (that is the major distinguishing feature making TAR syndrome different from other disorders featuring radial aplasia), thrombocytopenia, and a number[orpha.net]
Congestive Heart Failure
  • In acyanotic defects, congestive heart failure is the most common symptom.[aafp.org]
  • If untreated, it may lead to congestive heart failure.[icd9data.com]
  • Utility of B-type natriuretic peptide in differentiating congestive heart failure from lung disease in pediatric patients with respiratory distress. Pediatr Cardiol 2004;25:341–6. Savitsky E, Alejos J, Votey S.[emergencymedicinecases.com]
  • A large PDA can cause pulmonary hypertension and congestive heart failure if left untreated.[lecturio.com]
  • Symptoms of congestive heart failure such as rapid breathing, clammy sweating, and poor feeding often develop during the first week of life.[mottchildren.org]
Surgical Procedure
  • The surgical procedure when indicated consists of opening the pulley that causes the tight passage for the tendon.[columbiadoctors.org]
  • In addition, the companion VitalSource ebook contains more than 50 operative videos that have been edited and narrated to highlight the key steps in complex surgical procedures.[books.google.ro]
  • Surgical Procedures for Sphincteric Incontinence in the Male The Artificial Genitourinary Sphincter and Perineal Sling Procedures 427 Urothelial Tumors of the Bladder 432 NonMuscleInvasive Bladder Cancer Ta T1 and CIS 441 Management of Metastatic and[books.google.ro]
  • A brief discussion of pediatric murmurs follows, and this chapter concludes with a discussion of common surgical procedures for repairing congenital heart defects and associated complications.[accessmedicine.mhmedical.com]
  • The knowledge of such variation is important in surgical procedures related to the extra-hepatic biliary system.[jmedicalcasereports.biomedcentral.com]
Pulmonary Valve Stenosis
  • valve stenosis Tetralogy of Fallot Total anomalous pulmonary venous return Transposition of the great vessels Tricuspid atresia Truncus arteriosus Vascular ring Ventricular septal defect [ Read More ][icdlist.com]
  • valve stenosis Q22.2 Congenital pulmonary valve insufficiency Congenital pulmonary valve regurgitation Q22.3 Other congenital malformations of pulmonary valve Congenital malformation of pulmonary valve NOS Q22.4 Congenital tricuspid stenosis Tricuspid[apps.who.int]
  • Pulmonary valvuloplasty is indicated for a patient with critical pulmonary valve stenosis (defined as PS present at birth with cyanosis and evidence of PDA dependency), at any age post-neonatal with or without right ventricle dysfunction and a peak-to-peak[emjreviews.com]
Dyspnea
  • Interactive CardioVascular and Thoracic Surgery7: 1183–1185 [9] Shostak E, Sarwar A (2009) A 50-year-old woman with dyspnea, lower extremity edema, and volume loss of the right hemithorax.[eurorad.org]
  • However, some patients are symptomatic, reporting symptoms that include chest pain, palpitations, dyspnea, and syncope.[heartviews.org]
  • […] including: Lethargy and weakness Fast or labored breathing Bounding (very strong) pulse Tachycardia (a heart rate exceeding the normal resting rate) Cyanosis (a blue-ish skin color due to a lack of oxygen), primarily affecting the lower extremities Dyspnea[verywell.com]
  • Additionally, 1 patient had hemoptysis and dyspnea.[appliedradiology.com]
  • Heart failure increases metabolic demands and the associated dyspnea makes feeding more difficult.[msdmanuals.com]
Hemoptysis
  • Chest136(2): 628–632 [10] Bekoe S, Pellegrini RV, DiMarco RF Jr, Grant KJ, Woelfel GF (1993) Pneumonectomy for unremitting hemoptysis in unilateral absence of pulmonary artery.[eurorad.org]
  • Additionally, 1 patient had hemoptysis and dyspnea.[appliedradiology.com]
Heart Disease
  • Disease and Heart Disease in Pregnancy, Kaiser Foundation Hospitals, Panorama City, California, USA[books.google.com]
  • Abstract Newborn infants (NB) with cyanotic heart disease and reduced pulmonary flow are often ductus-dependent and improve with prostaglandin (PGE) infusion. However, PGE may fail if the ductus arteriosus is absent.[nature.com]
  • Posted in Cardiology - Clinical, cardiology -congenital heart disease, Cardiology -unresolved questions, cardiology congenital heart disese Tagged major aortopulmonary collaterals, mapcos, mapcos in tof why uncommon, pulmonary atresia and vsd, why pda[drsvenkatesan.com]
  • Congenital Heart Disease Research and Training Center Hektoen Institute for Medical Research Chicago[link.springer.com]
Heart Failure
  • Heart failure in infants and children has many causes other than congenital heart anomalies (see table Common Causes of Heart Failure in Children ).[msdmanuals.com]
  • The presence of significant tricuspid regurgitation also increases the risk of heart failure.[emedicine.medscape.com]
  • In acyanotic defects, congestive heart failure is the most common symptom.[aafp.org]
  • The Pink Infant: Pediatric Congestive Heart Failure (CHF) Heart failure usually presents with a ‘pink’ relatively well-perfused and oxygenated infant 1 to 6 months of age. Always consider CHF in the wheezing child![emergencymedicinecases.com]
Hypertension
  • Ann Thorac Surg79(4):1399–1402 [12] Toews WH, Pappas G (1983) Surgical management of absent right pulmonary artery with associated pulmonary hypertension. Chest84(4): 497–499[eurorad.org]
  • "Masked hypertension in young patients after successful aortic coarctation repair: impact on left ventricular geometry and function". Journal of Human Hypertension. 25 (12): 739–45. doi : 10.1038/jhh.2010.118.[en.wikipedia.org]
  • For a patent ductus arteriosus with a shunt large enough to cause symptoms of heart failure or pulmonary hypertension, closure should be done after medical stabilization.[msdmanuals.com]
  • Congenital heart disease Down's syndrome Eisenmenger's syndrome pulmonary arterial hypertension Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease (CHD), particularly in patients with left-to-right (systemic-to-pulmonary[err.ersjournals.com]
  • Not frequently, these fistulas themselves cause portal hypertension and high-output heart failure (, 29,, 30 ).[pubs.rsna.org]
Beak Nose
  • Reduced amniotic fluid volume causes increased pressure on the developing fetus, resulting in a sloped forehead, “parrot beaknose, shortened fingers, and hypoplasia of internal organs, particularly the gut and lungs.[web.duke.edu]

Workup

  • Further history and workup for deep vein thrombosis (DVT) and a hereditary hypercoagulable state failed to identify a cause for PE. The patient was discharged, but later on readmitted to the hospital with chest pain and palpitations.[heartviews.org]

Treatment

  • Simple superficial laser treatments have no effect on deep malformations that result in severe symptoms. Treatment is often performed in stages by combining the three treatment modalities ( Figure 12 ). Figure 11.[phlebolymphology.org]
  • Many children with congenital heart defects don't need treatment, but others do. Treatment can include medicines, catheter procedures, surgery, and heart transplants.[icdlist.com]
  • Depending of the type of anomaly, treatment will be recommended by the hand surgeon, the treatment may be surgical and non-surgical depending on each individual case.[columbiadoctors.org]
  • The ductus may close on its own, without treatment. If it doesn’t, treatments include: Medication Certain medications can constrict the artery, shrinking the duct and allowing it to close. The medication is given by IV over a period of a few days.[texaschildrens.org]

Prognosis

  • Noonan syndrome 4 non-syndromic anomalies, e.g. congenital cardiac anomalies 7 Treatment and prognosis The prognosis of fetuses with absent ductus venosus diagnosed at 11-13 weeks of gestation depends on the measurement of nuchal translucency thickness[radiopaedia.org]
  • Infants who require surgery early in life have a worse prognosis than those repaired at a later date.[emedicine.medscape.com]
  • Prognosis: • Good outcome with surgery.[slideshare.net]
  • […] intact ventricular septum has a relatively better prognosis. [10] 4 Conclusions APVS is a rare congenital heart disease and often associated with TOF, but has an overall poor prognosis.[journals.lww.com]

Etiology

  • Etiology The etiology and inheritance pattern remain unclear. In all patients, a deletion on chromosome 1q21.1 has been found. However, at present, it is still unclear how this relates to the cause in TAR syndrome.[orpha.net]
  • Epidemiology and Natural History 482 Evaluation and Nonsurgical Management of Benign Prostatic Hyperplasia 486 Minimally Invasive and Endoscopic Management of Benign Prostatic Hyperplasia 493 Retropubic and Suprapubic Open Prostatectomy 496 Epidemiology Etiology[books.google.ro]
  • Etiology and Pathophysiology Often, PDA has no clear-cut cause; however, it is believed that genetic and environmental factors may be involved.[news-medical.net]
  • Etiology of Patent Ductus Arteriosus (PDA) The etiological factors of PDA can be assessed as following: The exact etiology of PDA is unknown but familial cases have been reported. Genetic causes are not established.[lecturio.com]
  • The neonatal vanishing kidney: congenital and vascular etiologies. J Urol 2004;172:317-8. [ PUBMED ] [ FULLTEXT ] 5. McCallum TJ, Milunsky JM, Munarriz R, Carson R, Nejad HS, Oates RD.[sjkdt.org]

Epidemiology

  • […] and Natural History 482 Evaluation and Nonsurgical Management of Benign Prostatic Hyperplasia 486 Minimally Invasive and Endoscopic Management of Benign Prostatic Hyperplasia 493 Retropubic and Suprapubic Open Prostatectomy 496 Epidemiology Etiology[books.google.ro]
  • Summary Epidemiology It occurs in less than 1 per 100,000 live births, equally affecting both sexes.[orpha.net]
  • Paradoxical emboli Complication Instead of eg DVTs going into lung May be able to go through ASD into systemic circulation and cause stroke etc Patent Ductus Ateriosus Spiral smooth muscle Epidemiology Term - 1:2000 [10% of congenital heart defects] inc[alancam.com]
  • An epidemiology paper from Carleton covering the period between 1941–58 found only 41 families where the same cardiac defect occurred in two family members and of these only 17 had patent arterial duct [ 12 ].[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • (Click picture to show/hide bloodflows) Pathophysiology For blood to exit the right ventricle, it must either return to the right atrium via the tricuspid valve or drain into the coronary circulation via sinusoids.[vhlab.umn.edu]
  • Cutaneous Continent Urinary Diversion 459 Orthotopic Urinary Diversion 465 Genital and Lower Urinary Tract Trauma 468 Lower Urinary Tract Calculi 471 Development Molecular Biology and Physiology of the Prostate 476 Benign Prostatic Hyperplasia Etiology Pathophysiology[books.google.ro]
  • Etiology and Pathophysiology Often, PDA has no clear-cut cause; however, it is believed that genetic and environmental factors may be involved.[news-medical.net]
  • Distinct clinical presentations are discussed further in later sections, including the pathophysiology, clinical features and treatment, and individual defects within each group.[accessmedicine.mhmedical.com]
  • The current European Society of Cardiology guidelines provide an anatomical–pathophysiological classification of the congenital left-to-right shunts associated with PAH [ 3 ].[err.ersjournals.com]

Prevention

  • Esophageal atresia prevents the normal passage of food and saliva to the stomach. A ____ is an abnormal opening between the trachea and esophagus. tracheoesophageal fistula[quizlet.com]
  • After closure using catheter based procedures, preventive antibiotics are gives for 6 months to prevent infection.[fairview.org]
  • While they can't be prevented, many treatments are available for the defects and related health problems.[m.kidshealth.org]
  • For those with experiencing pulmonary hypertension, a fluid restricted diet may be prescribed to prevent the excessive buildup of fluids which can place undue pressure on the heart. In some cases, surgery may be required.[verywell.com]
  • Prevention of bleeding and hemorrhage in the first years of life is essential to reduce the clinically significant morbidity. Severe thrombocytopenia may require platelet transfusions.[orpha.net]

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