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TAPVR is constitute in 1 - 1.5 % of all patients with congenital cardiac abnormality ; whereas , PAPVR is found at a rate of 0.6 % in postmortem examination findings(1 ) .
The former and late deathrate rates for simple TAPVR are around 10 % and 4 % ; whereas , for PAPVR they are 0 % and < 1%(1 ) .
What Are The Differences Between PAPVR & TAPVR?
Partial Anomalous Pulmonary Venous Return ( PAPVR ) and Total Anomalous Pulmonary Venous Return ( TAPVR ) are both abnormality of pneumonic venous origin flow , which are rare congenital essence deformities . In these , the pulmonary vein do not join to the left-hand atrium instead run out oxygenise stock to the veracious side of the heart . If all four pulmonary venous blood vessel are abnormally connected then it is termed as TAPVR and if 3 or less than 3 pulmonary vein are anomalous then it is term as PAPVR . TAPVR is more uncouth in males whereas PAPVR is more commonly seen in female person .
TAPVR can be classify into four different type namely :
The classification of PAPVR include :
PAPVR With Sinus Venosus Atrial Septal Defect ( 80 - 90 % Of The Cases):Normally , the correct upper or center pulmonary nervure enfeeble into the superscript vein cava or the correct atrium .
separated PAPVR or PAPVR Without Atrial Septal Defect : Usually , a rare phenomenon and mainly the correct upper pulmonary vena drain into the superior vena cava .
PAPVR With Complex Congenital Heart Disease Or Heterotaxia : The right pneumonic vein drain into the right atrium and associated with obstruction and pulmonary arterial hypoplasia
Scimitar Syndrome : The anomalous connection of the right pulmonary nervure to the substandard vein cava with right lung segregation , lung hypoplasia , and dextrapositioning of the heart to the correct side(2 ) .
Clinical Presentation Of TAPVR & PAPVR
Since both are innate disorder and present by birth , neonates with TAPVR are symptomatic from the beginning ; whereas , patients with PAPVR become symptomatic in middle age or stay symptomless throughout life depending on the number of pneumonic veins involve .
TAPVR patients present with cyanosis , brusqueness of breath , diaphoresis ( sweating ) on feeding , loser to uprise , liver enlargement , correct heart failure , systemic hypotension , metabolic acidosis , andpulmonary high blood pressure . TAPVR patient with obstruction additionally present with serious cyanosis and shortness of breath , tachycardia , and sometimes acute respiratory failure along with the above - mentioned symptoms(1 ) .
The symptoms of PAPVR are usually mild or the affected role are completely asymptomatic . fry are usually asymptomatic , except cardiac mussitation or intolerance to do . Adults may exhibit with gruffness of hint , arrhythmias , peripheral dropsy , right nerve failure , lung infections , or haemoptysis in case of pulmonary venous disease(2 ) .
Management Of TAPVR & PAPVR
TAPVR and PAPVR both need to be managed very differently . TAPVR specially the obstruct type needs to be surgically objurgate within hours to Clarence Day of aliveness ; whereas , PAPVR can be managed medically without the want for immediate surgical procedure . TAPVR patients can be stabilized medically with mechanically skillful ventilation , inotropic drugs , nitric oxide inspiration preoperatively , prostaglandin , and ECMO ( extracorporeal tissue layer oxygenation ) . The surgical process of TAPVR is aspire at separating all the anomalous pulmonic vein contact and connecting the pulmonary vein to the left over atrium(1 ) .
Generally , the medical prognosis of TAPVR patients is subordinate on the academic degree of obstruction of pulmonary vein and other defects link up with it . The early and tardy death rate rates for simple TAPVR are around 10 % and 4 % ; for TAPVR with pneumonic impedimenta are 17 and 11 % , respectively ; for unobstructed affected role , they are 4 % and 6 % , severally ; TAPVR connect with other cardiac abnormalities are 17 % and 11 % ; whereas , for PAPVR they are 0 % and < 1%(1 ) .
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