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| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Infundibular Pulmonary Stenosis|Infundibular Pulmonary Stenosis]]'''<ref name="pmid26855934">{{cite journal| author=Burt T, Sharma P, Mittal S| title=Research Question, Study Design and Continuous Research Education and Training Exercises (CREATE) Program. | journal=J Clin Prev Cardiol | year= 2012 | volume= 1 | issue= 1 | pages= 35-43 | pmid=26855934 | doi= | pmc=4739791 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26855934  }}</ref><ref name="pmid17700348">{{cite journal| author=Scales LH, McEwen IR, Murray C| title=Parents' perceived benefits of physical therapists' direct intervention compared with parental instruction in early intervention. | journal=Pediatr Phys Ther | year= 2007 | volume= 19 | issue= 3 | pages= 196-202 | pmid=17700348 | doi=10.1097/PEP.0b013e3180cabc57 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17700348 }}</ref>
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| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Infundibular Pulmonary Stenosis|Infundibular Pulmonary Stenosis]]'''<ref name="pmid8282434">{{cite journal| author=Shyu KG, Tseng CD, Chiu IS, Hung CR, Chu SH, Lue HC | display-authors=etal| title=Infundibular pulmonic stenosis with intact ventricular septum: a report of 15 surgically corrected patients. | journal=Int J Cardiol | year= 1993 | volume= 41 | issue= 2 | pages= 115-21 | pmid=8282434 | doi=10.1016/0167-5273(93)90150-f | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8282434 }}</ref>
 
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* Asymptomatic, he hypertrophied right ventricle can maintain adequate flow across the obstruction even in severe stenosis
 
* Asymptomatic, he hypertrophied right ventricle can maintain adequate flow across the obstruction even in severe stenosis
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Revision as of 22:30, 26 March 2020

Ventricular septal defect Microchapters

Home

Patient Information

Overview

Classification

Pathophysiology

Causes

Differentiating Ventricular Septal Defect from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Electrocardiogram

Chest X Ray

CT

MRI

Echocardiography

Cardiac Catheterization

Treatment

Medical Therapy

Surgery

Ventricular septal defect post-surgical prognosis

ACC/AHA Guidelines for Surgical and Catheter Intervention Follow-Up

Prevention

ACC/AHA Guidelines for Reproduction

Cost-Effectiveness of Therapy

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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Leida Perez, M.D. Associate Editor(s)-In-Chief: Keri Shafer, M.D. [2], Priyamvada Singh, MBBS

Overview

Differentiating Ventricular Septal Defect from other Disorders



Disease Signs and Symptoms Association Diagnostic modality Management Prognosis
VSD[2]
  • Shortness of breath
  • Tachypnea
  • Palpitation
  • Paleness
  • Failure to gain weight
  • Sweating while feeding
  • Frequent respiratory infections
  • Fainting
  • Chest pain
  • Cyanosis
  • Down syndrome
  • ventricular septal defects in association with other malformation syndromes:
  • fetal alcohol syndrome
  • trisomy syndromes
  • fetal hydantoin syndrome
  • postrubella infection (German measles)
  • maternal phenylketonuria (PKU)
  • Tetralogy of Fallot
  • Holt-Oram Syndrome
  • FG Syndrome
  • Genitopalatocardiac Syndrome
  • Fryns Syndrome
  • certain forms of Dandy-Walker Syndrome
  • Cardiomyopathy-Hypogonadism-Collagenoma Syndrome
  • Familial Idiopathic Cardiomyopathy
  • Simpson Dysmorphia Syndrome
  • Fetal Alcohol Syndrome
  • DiGeorge Syndrome
  • various Trisomy Syndromes
  • maternal alcoholism
  • maternal ingestion of phenylhydantoin
  • postrubella infection
  • maternal Phenylketonuria
  • Echocardiogram
  • Electrocardiogram (ECG)
  • Chest X-ray
  • Cardiac catheterization
  • Pulse oximetry
  • Small VSD: Observe, symptomatic treatment until spontaneous closure.
  • Surgical repair:
  • During first year of life
  • Immediately if severe symptoms present
  • Small VSD to prevent complications related to their locations, such as heart valves damage
  • Nutritional support or tubal feeding if VSD causing tiredness of the infant during feeding
  • Surgical repair
  • Catheter procedure
  • Hybrid procedure (surgical and catheter-based techniques)
  • Small VSD usually closes spontaneously by 18 months. Up to 75% are closed by the age of 10.
  • Medium to large VSDs often become smaller but remain patent and allow shunting of blood and eventual development of Eisenmenger’s syndrome and heart failure.
  • If large defects are not corrected before pulmonary hypertension develops the prognosis is poor.
  • Patients with the Eisenmenger’s syndrome have an average life expectancy of 33 years.
  • Surgical correction provide a better outcome.
Atrioventricular septal defect[3]
  • Cyanosis mild or absent
  • Congestive cardiac failure
  • Right ventricular impulse
  • Increased pulmonic component second heart sound
  • Variable ejection systolic murmur, apical mid‐diastolic murmur (in large left to right shunt), pansystolic murmur (with atrioventricular valve regurgitation)
  • Subaortic stenosis
  • Ventricular hypoplasia
  • Tetralogy of Fallot
  • Atrial isomerism
  • Antenatal ultrasound anomaly scanning (four‐chamber view)
  • Postnatal diagnosis:
  • ECG
  • Chest radiograph
  • Echocardiogram
  • Magnetic resonance imaging
  • Angiography
Medical treatment

CHF:

  • diuretics and vasodilator such as captopril
  • digoxin (controversial)

feeding difficulties and failure to thrive

  • nasogastric tube to


Without surgery the natural history of complete AVSD, only 4% survival beyond 5 years old
Atrial septal defect[4][5][6][7]
  • Asymptomatic
  • Soft, systolic ejection murmur over the pulmonic area (second intercostal space) combined with a wide, fixed splitting of S2
  • Large defects:
  • Exercise intolerance
  • Cardiac dysrhythmias
  • Palpitations
  • Increased incidence of pneumonia, pulmonary hypertension and increased mortality
  • Down syndrome
  • Noonan syndrome
  • Ellis van-Creveld syndrome
  • Opitz syndrome
  • Costello syndrome,
  • Chondroectodermal dysplasia
  • Rubella
  • Holt-Oram syndrome
  • Hurler syndrome
  • Echocardiogram
  • Chest X-ray
  • Electrocardiogram
  • Cardiac catheterization
  • MRI
  • CT scan
  • Medical monitoring
  • echocardiograms and annual exam to check for complications, such as pulmonary hypertension, arrhythmias, heart failure or valve problems
  • Medications
  • Surgery
  • Follow-up care
  • The surgical mortality rate increases with increasing age and pulmonary artery pressures.
  • Patients < 45 years without heart failure and with systolic pulmonary artery pressures less than 60 mm Hg have a mortality rate of surgical repair < 1%
Patent Ductus Arteriosus (PDA)[8] In adults is usually a coincidental finding during physical examination or echocardiography screening.
  • Atypical continuous murmur which can be heard at the higher left sternal edge.
  • May be associated with a wide pulse pressure due to the runoff to the pulmonary circulation.
  • Chest Radiograph
  • Electrocardiogram
  • Echocardiogram
  • Magnetic Resonance Imaging and Computed Tomography
  • Cardiac Catheterization
  • Transcatheter Closure
  • Surgical Therapy
  • Prognosis depends on the size and magnitude of the shunt and the status of the pulmonary vasculature
  • Small PDA has normal prognosis
  • Large PDA with significant left heart volume overload:
  • Congestive Heart Failure
  • Hypertensive Pulmonary Vascular Disease
  • Endarteritis
  • Aneurysm of Ductus Arteriosus
  • recurrent laryngeal nerve paralysis
Infundibular Pulmonary Stenosis[9]
  • Asymptomatic, he hypertrophied right ventricle can maintain adequate flow across the obstruction even in severe stenosis
  • Dyspnea
  • Chest pain
  • Palpitation on effort
  • Epigastric pain (exercise-induced right ventricular failure and hepatic congestion)
  • Presyncope or syncope on efforts (When the right ventricle fails to maintain adequate cardiac output)
  • Sudden death (severe stenosis even when asymptomatic)

References

  1. LAMBERT EC, KELSCH JV, VLAD P (1963). "Differential diagnosis of ventricular septal defect in infancy: a common problem". Am J Cardiol. 11: 447–51. doi:10.1016/0002-9149(63)90003-1. PMID 13928242.
  2. Cleves MA, Hobbs CA, Cleves PA, Tilford JM, Bird TM, Robbins JM (2007) Congenital defects among liveborn infants with Down syndrome. Birth Defects Res A Clin Mol Teratol 79 (9):657-63. DOI:10.1002/bdra.20393 PMID: 17696161
  3. Craig B (2006). "Atrioventricular septal defect: from fetus to adult". Heart. 92 (12): 1879–85. doi:10.1136/hrt.2006.093344. PMC 1861295. PMID 17105897.
  4. El-Segaier M, Pesonen E, Lukkarinen S, Peters K, Ingemansson J, Sörnmo L; et al. (2006). "Atrial septal defect: a diagnostic approach". Med Biol Eng Comput. 44 (9): 739–45. doi:10.1007/s11517-006-0094-5. PMID 16941100.
  5. Yoshihara K, Ozawa T, Sakuragawa H, Fujii T, Kawasaki M, Shiono N; et al. (1999). "[Noonan syndrome associated with atrial septal defect, pulmonary stenosis, and completely unroofed coronary sinus without LSVC: a case report]". Kyobu Geka. 52 (2): 134–7. PMID 10036874.
  6. Geva T, Martins JD, Wald RM (2014). "Atrial septal defects". Lancet. 383 (9932): 1921–32. doi:10.1016/S0140-6736(13)62145-5. PMID 24725467.
  7. Goldberg JF (2015). "Long-term Follow-up of "Simple" Lesions--Atrial Septal Defect, Ventricular Septal Defect, and Coarctation of the Aorta". Congenit Heart Dis. 10 (5): 466–74. doi:10.1111/chd.12298. PMID 26365715.
  8. Schneider DJ, Moore JW (2006). "Patent ductus arteriosus". Circulation. 114 (17): 1873–82. doi:10.1161/CIRCULATIONAHA.105.592063. PMID 17060397.
  9. Shyu KG, Tseng CD, Chiu IS, Hung CR, Chu SH, Lue HC; et al. (1993). "Infundibular pulmonic stenosis with intact ventricular septum: a report of 15 surgically corrected patients". Int J Cardiol. 41 (2): 115–21. doi:10.1016/0167-5273(93)90150-f. PMID 8282434.




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