Cyanosis, when present for > 6 mo, may result in clubbing. [merckmanuals.com]

Tricuspid atresia: complete absence of the tricuspid valve; an ASD must be present to maintain blood flow. [patient.info]

Slide 1 Everything You Need To Know About Content Marketing Hana Abaza, Director of Marketing at Uberflip @hanaabaza 1 WHY IT’S HOT Authoring – Create your content.… All About Beer PowerPoint Presentation Text Me: Do's & Don'ts of Presentation Design [dokumen.tips]

Presentation Children born with pulmonary atresia and ventricular septal defect (PA-VSD) may have unpredictable presentations owing to the variability of the lesion. The age at presentation may vary depending on the amount of pulmonary blood flow. [emedicine.medscape.com]

Present On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery [icd10data.com]

• Hypoxemia

  If intervention is needed for hypoxemia within the first 4 to 8 wk of life, a modified Blalock-Taussig shunt (connection of a systemic and a pulmonary artery by a synthetic tube) is done. [merckmanuals.com]

  The resulting hypoxemia manifests clinically as cyanosis, which may occur as acute, life-threatening episodes. Further symptoms include failure to thrive, characteristic heart murmurs, and symptoms of heart failure. [amboss.com]

  […] development of severe tricuspid valve incompetence, there is no resulting congestive heart failure (with pleural and pericardial effusion and ascites) and the incidence of fetal death is low. 9,10 The clinical manifestations in the newborn are cyanosis, hypoxemia [revespcardiol.org]

  […] peripheral arteries is quite variable ranging from mild stenosis to complete atresia and account for the variability of clinical symptoms those with severe pulmonic obstrucion present in the first days of life as the ductus closes with extreme cyanosis and hypoxemia [atlases.muni.cz]

• Cyanotic Congenital Heart Disease

  Management of Cyanotic Congenital Heart Disease The following is a brief summary of acute and long term management of cyanotic congenital heart disease. [learn.pediatrics.ubc.ca]

  Significant amounts of blood shunting from right to left without traversing the lungs causes cyanotic congenital heart disease. [patient.info]

  […] endocarditis for all patients with unrepaired cyanotic congenital heart disease The “5 Ts” of cyanotic CHDs: T etralogy of Fallot, T ransposition of the great vessels, T ricuspid atresia, T otal anomalous pulmonary venous return, and T runcus arteriosus [amboss.com]

  Background Pulmonary atresia with ventricular septal defect (PA-VSD) is a cyanotic congenital heart disease characterized by underdevelopment of the right ventricular (RV) outflow tract (ie, subpulmonary infundibulum) with atresia of the pulmonary valve [emedicine.medscape.com]

• Developmental Disorder

  […] vascular disorders, pulmonary hemosiderosis, immunodeficiency syndromes, or hypersensitivity peumonitis. [ojrd.biomedcentral.com]

• Thrombosis

  […] e.g. post-repair of anomalous pulmonary venous return Complications Recognized complications include pulmonary hypertension - pulmonary hypertensive crisis recurrent intercurrent pulmonary infection hemoptysis See also pulmonary vein pulmonary vein thrombosis [radiopaedia.org]

  […] parenchymal lesions have also been reported in diseases, such as Sjogren’s syndrome and systemic sclerosis. 1 Additional rare causes of unilateral lesions include pulmonary circulatory diseases, such as proximal PA blockage (eg, PA sarcoma), venous thrombosis [thorax.bmj.com]

• Acrocyanosis

  Acrocyanosis (cyanosis only in peripheral parts), commonly seen with cold environments or after bathing, is always a normal finding and is not a true cyanosis. Long standing cyanosis results in digital clubbing. [learn.pediatrics.ubc.ca]

Yet the diagnostic workup of pulmonary venous stenosis is elusive, which is why the different diagnostic procedures to detect pulmonary venous stenosis are discussed here. [err.ersjournals.com]

Treatment Reimplantation of coronary arteries Aortocoronary bypass [amboss.com]

After treatment A good result from a balloon valvoplasty or surgery usually means that further treatment is not required. [chd-uk.co.uk]

In cases where the right ventricle is normal in size, treatment of critical stenosis or atresia is performed soon after birth. Treatment options include open-heart surgery and interventional cardiac catheterization. [luriechildrens.org]

The prognosis in certain subsets of patients remains grim in spite of advances in treatment. Larger studies using newer modalities of treatment would help to improve prognosis in the difficult to treat subsets. [medresearch.in]

Prognosis: Cardiac failure is never seen in fetal life as well as postnatally. [sonoworld.com]

[…] the prognosis has greatly improved over the years for those with pulmonary atresia. [en.wikipedia.org]

The prognosis in certain subsets of patients remains grim in spite of advances in treatment. Larger studies using newer modalities of treatment would help to improve prognosis in the difficult to treat subsets. [medresearch.in]

Conclusions: Surgical recanalization of pulmonary vein atresia is possible, but prognosis remains poor. [link.springer.com]

Prognosis The prognosis for children with congenital heart disease has improved dramatically over the last few decades. [patient.info]

In most cases the etiology is unknown, probably multifactorial. [atlases.muni.cz]

References: [19] [1] [1] [20] [2] [21] [22] [23] [24] [25] Truncus arteriosus Definition Failed separation of aorta and pulmonary artery during development → single trunk that receives output from both ventricles Etiology Failure of cardiac neural crest [amboss.com]

Pulmonary vein stenosis: Etiology, diagnosis and management. [wjgnet.com]

^ "Pulmonary Atresia With Intact Ventricular Septum: Background, Pathophysiology, Etiology". 2018-06-07. ^ a b c "Pulmonary Atresia With Ventricular Septal Defect: Background, Epidemiology, Etiology". [en.wikipedia.org]

Etiology Pediatr Cardiol 2016 Jan;37(1):125-30. Epub 2015 Aug 14 doi: 10.1007/s00246-015-1249-7. PMID: 26271471 Bonello B, Trivedi KR, Fraisse A Catheter Cardiovasc Interv 2015 Oct;86(4):E190-3. Epub 2015 May 27 doi: 10.1002/ccd.25920. [ncbi.nlm.nih.gov]

References: [15] Tricuspid valve atresia Definition Absent or rudimentary tricuspid valve, resulting in no blood flow between the right atrium and the right ventricle Epidemiology Third most common cyanotic heart defect Almost always accompanied by ASD [amboss.com]

^ a b c "Pulmonary Atresia With Ventricular Septal Defect: Background, Epidemiology, Etiology". August 2018. ^ "Research | Congenital Heart Defects | NCBDDD | CDC". www.cdc.gov. [en.wikipedia.org]

Epidemiology The reported total birth prevalence of congenital heart disease in Europe is 8.2 per thousand live births which is higher than reported birth prevalence in the USA (6.9 per thousand live births). [patient.info]

Epidemiology Frequency The best estimates of the relative frequency of pulmonary atresia with ventricular septal defect are 2.5-3.4% of all congenital cardiac malformations with a prevalence of 0.07 per 1000 live births. [2, 3] Pulmonary atresia with [emedicine.medscape.com]

Some disease registries, like the cancer registry, closely resemble public health surveillance systems and have epidemiologic value. Renal agenesis/hypoplasia: Absence or incomplete development of the kidney. [fbdr.org]

Overview of cyanotic congenital heart defects General pathophysiological processes Heart defects may lead to the formation of connections between the right and the left heart ( see “Pathophysiology” in acyanotic congenital heart defects ). [amboss.com]

^ "Pulmonic Valve Anatomy: Overview, Pathophysiologic Variants". 2017-09-14. ^ "Congenital Heart Defects: MedlinePlus". [en.wikipedia.org]

Pathophysiology To understand the pathophysiology of haemoptysis in cases of pulmonary venous stenosis, it is important to know that both the pulmonary and the bronchial circulation drain via the pulmonary veins into the left atrium. [err.ersjournals.com]

Each of these types can be subdivided based on the degree of obstruction to pulmonary blood flow: A—pulmonary arteries are atretic B—pulmonary stenosis or hypoplasia C—no obstruction to pulmonary flow (Click picture to show/hide bloodflows) Pathophysiology [vhlab.umn.edu]

Classification Classification into five groups based on pathophysiology has been suggested as it would be helpful in planning management [ 1 ]. [patient.info]

Endocarditis Prevention Antibiotics to prevent endocarditis are needed before certain dental procedures. See the section on Endocarditis for more information. [heart.org]

One-way valves are present within the heart to prevent blood from flowing back into it in the wrong direction. [savetheheartbeat.org]

Prevention & Risk Assessment Prevention & Risk Assessment What causes pulmonary stenosis? Congenital pulmonary stenosis occurs due to improper development of the pulmonary valve in the first eight weeks of fetal growth. [childrensnational.org]

References Mai CT, Riehle-Colarusso T, O’Halloran A, Cragan JD, Olney RS, Lin A, Feldkamp M, Botto LD, Rickard R, Anderka M, Ethen M, Stanton C, Ehrhardt J, Canfield M; National Birth Defects Prevention Network. [cdc.gov]

Prevention Although many forms of congenital heart disease are not currently preventable, the avoidance of known risk factors such as drugs and alcohol during pregnancy will help to reduce the risk. [patient.info]