Absent pulmonary valve: Difference between revisions

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Latest revision as of 18:55, 14 March 2016

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and Related Keywords: Absent pulmonary valve syndrome, APVS, Congenital absence of the pulmonary valve, Pulmonary valve agenesis

Absent pulmonary valve is a rare congenital heart defect. This anomaly is a combination of poorly formed pulmonary valve tissue and significant pulmonary valve insufficiency. This may cause pulmonary artery dilatation. Massive dilatation of pulmonary arteries may lead to extrinsic compression of bronchial airway. ^[1] ^[2] ^[3]

Possible associated abnormalities include;

Diagnosis

Symptoms can vary depending on associated defects, and may include:

Diagnostic Tests

Electrocardiogram
Chest x-ray
Echocardiogram
Computed tomography of the heart
Magnetic resonance imaging (MRI) of the heart

Treatment

Infants who have respiratory symptoms should have surgery as soon as possible. Others without symptoms should have surgery within the first 3-6 months of life.

Complications

References

↑ Park, MK. Park: Pediatric Cardiology for Practitioners. 5th ed. Philadelphia, Pa: Mosby; 2008.
↑ Nlke L, Azakie A, Anagnostopoulos PV, Alphonso N, Karl TR. The Lecompte maneuver for relief of airway compression in absent pulmonary valve syndrome. Ann Thorac Surg, 2006;81:1802-1807.
↑ Brown JW, Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW. Surgical treatment of absent pulmonary valve syndrome associated with bronchial obstruction. Ann Thoracic Surg, 2006; 82: 2221-2226.

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v t e Congenital malformations and deformations of circulatory system (Q20-Q28, 745-747)
Cardiac chambers and connections	Persistent truncus arteriosus - Double outlet right ventricle (Taussig-Bing syndrome) - Transposition of the great vessels (dextro, levo)
Cardiac septa	Ventricular septal defect - Atrial septal defect (Lutembacher's syndrome) - Atrioventricular septal defect (Ostium primum) - Tetralogy of Fallot - Eisenmenger's syndrome
Right: pulmonary and tricuspid valves	pulmonary valves (stenosis, insufficiency) - tricuspid valves (stenosis, atresia) - Ebstein's anomaly
Left: aortic and mitral valves	aortic valves (stenosis, insufficiency, bicuspid) - mitral valves (stenosis, regurgitation) - Hypoplastic left heart syndrome
Other congenital malformations of heart	Dextrocardia - Levocardia - Cor triatriatum
Great arteries	aorta (Patent ductus arteriosus, Aortic coarctation, Interrupted aortic arch, Overriding aorta, Aneurysm of sinus of Valsalva, Vascular ring) - Pulmonary atresia
Great veins	Persistent left superior vena cava - Total anomalous pulmonary venous connection - Scimitar syndrome
Other	Arteriovenous malformation (Cerebral arteriovenous malformation)
See also non-congenital conditions (I, 390-459)

v t e Electrocardiography
Overview	History of the EKG • EKG interpretation basics • Normal sinus rhythm
EKG Complexes	P wave • QRS complex • ST Segment • T wave • T wave alternans • Tombstone T wave • U wave Osborn wave • H wave • K wave • Delta wave NSSTW changes
EKG Intervals	PR Interval • QRS Interval • ST Interval • QT Interval
Conduction System & Bradycardia	Cardiac pacemaker • SA node • AV node• Bundle of His • Purkinje fibers • Sinus bradycardia • First Degree AV Block • Second Degree AV Block • Complete or Third-Degree AV Block • Concealed conduction • AV Junctional Rhythms • LBBB • LAHB • LPHB • RBBB • Trifascicular block
Atrial Arrhythmias	Sinus tachycardia • Premature Atrial Contractions (PACs) • Ectopic Atrial Rhythm • Paroxysmal Atrial Tachycardia (PAT) • Paroxysmal Atrial Tachycardia (PAT) with Block • Multifocal Atrial Tachycardia (MAT) • Atrial Flutter • Atrial Fibrillation • Wandering atrial pacemaker
Ventricular Arrhythmias	Differential Diagnosis of Tachycardia with a Wide QRS Complex • Accelerated Idioventricular Rhythm • Ventricular Parasystole • Premature Ventricular Contractions (PVCs) • Ventricular tachycardia • Ventricular Fibrillation • Sudden cardiac death
EKG Abnormalities in Disease States	Hypertrophy & Dilatation • Right atrial enlargement • Left atrial enlargement • Biatrial enlargement • Left Ventricular Hypertrophy • Right Ventricular Hypertrophy • Biventricular Hypertrophy • Acute myocardial infarction • NSTEMI • STEMI • Tombstone ST elevation • Right ventricular myocardial infarction • Atrial infarction Pre-excitation Syndromes • Wolff-Parkinson-White Syndrome • Lown Ganong Levine Syndrome • Mahaim Type Preexcitation Cardiomyopathies • Arrhythmogenic Right Ventricular Dysplasia • Dilated Cardiomyopathy • Hypertrophic Cardiomyopathy Drug Effects on the EKG • Adenosine • β-blockers • Digitalis • Quinidine • Procainamide • Disopyramide • Lidocaine • Tocainide and Mexiletine • Phenytoin • Encainide, Flecainide and Propafenone • Amiodarone • Bretylium • Ca Channel Blockers • Phenothiazines • Tricyclic Antidepressants • Lithium Congenital Heart Disease • Dextrocardia • Atrial Septal Defect • Ventricular Septal Defect • Tetralogy of Fallot • Conjoined Twins or Siamese Twins • Congenital heart block Electrolyte Disturbances • Hyperkalemia • Hypokalemia • Hypercalcemia • Hypocalcemia • Nonspecific Changes Other Heart Diseases • Pericarditis • Myocarditis • Tamponade • Heart Transplantation • Sick Sinus Syndrome • Long QT Syndrome Inherited Disease • Brugada Syndrome Systemic Diseases • CNS Disease • Cardiac Tumors Heart Transplantation • EKG Changes in patient with Heart Transplantation Exogenous Effects • Hypothermia • Chest Trauma • Insect Bites • Electric Injuries
Technical Issues and Potential Errors in Interpretation	Artifacts • Lead Placement Errors • The EKG in a Patient with a Pacemaker • EKG in athletes