Download CME: Total Anomalous pulmonary venous Drainage(TAPVD)

CME: Total Anomalous pulmonary venous Drainage(TAPVD)
Incidence: The incidence of total anomalous pulmonary venous drainage is 0.008% of live
births, 2.2% in patients with congenital heart disease.
What is the TAPVD : Total anomalous pulmonary venous return is a congenital anomaly in
which there is no connection between the pulmonary veins and the left atrium; the pulmonary
veins instead connect directly into the right atrium or to a systemic vein (Innominate, superior
vena cava (SVC), Azygus, inferior vena cava ( IVC) , hepatic vein or portal vein) by an
alternative pathway (vertical vein). Because all venous blood returns to the right atrium, the
survival of baby depends upon the obligatory connection between the left and right atria.
Classification of TAPVD : Darvin et al ( 1957) :
Type-I: TAPVR involves drainage into the systemic venous system via a left-side vertical
vein (an embryologic remnant of the left cardinal system), which drains into the left
brachiocephalic vein or directly into the right superior vena cava.
It accounts for 46% of patients who have TAPVR.
Incidence: 46% of total cases
Type II : It involves drainage directly into the heart, usually via the coronary sinus or directly
into the right atrium.
Incidence- 26% of total cases.
Type III : It involves drainage via a descending vein that courses below the diaphragm and
joins the inferior vena cava, hepatic veins, or portal system. This type typically features
obstruction of the pulmonary venous return and clinically present during the initial days of
extrauterine life.
Incidence: 24 % of total cases.
Type IV, It is rare, involves multiple sites of drainage.
Incidence: approx 5% of total cases.
Clinical features :
Onset of symptoms: Most patients develop symptoms within the 1st year of life: tachypnea,
feeding difficulties, frequent respiratory infections, and failure to thrive.
Early onset of symptom: Those patients who have pulmonary venous obstruction may present
urgently within the 1st few days of life with tachypnea, cyanosis, and heart failure.
Classical Clinical Findings :
Unobstructed TAPVD : Classical findings of ASD - wide and fixed split of second heart sound with
ejection RV S3 and ejection systolic murmur in an infant presenting with congestive heart failure and
borderline cyanosis must raise the suspicion of TAPVD.
Obstructed TAPVD : Usually in TAPVD pulmonary venous blood takes a longer course to reach left
atria. This pathway can be restrictive at places like at level of interatrial connection or draining ascending
or descending vertical vein leading to increase in pulmonary venous pressure and decrease in systemic
cardiac output.
This diagnosis must be suspected in a very sick cyanosed (borderline) neonate with CHF who has a
normal split of second heart sound with loud P2 with RVS3, ESM .
Chest X-Ray : The chest radiographs of patients who have unobstructed types of TAPVR
typically exhibit right atrial and right ventricular hypertrophy with increased pulmonary blood
flow. In patients whose return is to the left brachiocephalic vein, there may be a characteristic
enlargement of the superior mediastinum, bilaterally in a figure-8 or snowman shape. An
electrocardiogram will usually show right-axis deviation with right atrial P-wave abnormality
and right ventricular hypertrophy.
Obstructed TAPVC presents with relatively normal cardiac size and features of pulmonary
venous hypertension, ground glass opacity.
Echocardiography: Two-dimensional echocardiography has been shown to establish the
diagnosis accurately.
CT Angio/ conventional catheterization : may be necessary if echocardiography is
inconclusive in determining the site(s) of the pulmonary venous connections.
Treatment: Precise description of the drainage sites is important in determining the suitable
surgical approach. The diagnosis of TAPVR is an indication for open heart surgery. If the
condition remains untreated, it is usually fatal before 1 year of age. Since 1963, the standard
incision has been a median sternotomy.
Surgical Mortality and Morbidity Due to better diagnostic capabilities and to improvements in
surgical techniques, operative death has decreased from 15.8% in 1984 to as low as 0 in a later
case series. The repair of TAPVR has become a surgical procedure with very low risks of
morbidity and death.
Long-term Outcome of TAPVD repair : The repair of supradiaphragmatic TAPVR in
infants, children, and even adolescents is followed a normal life similar to that after the closure
of a nonrestrictive ostium secundum atrial septal defect and a long-term survival is expected in
most patients. Michielon G, reported a postsurgical survival rate of 87.3% for patients at 18
years of age, with no difference according to anatomic type of TAPVR or surgical technique.
Complication:, Major expected morbidities observed in a post op patients are –
1. Post-repair pulmonary venous obstruction (around 11%) : May need reintervention.
2. Pulmonary vein stenosis: May pre exist but may go undetected before the surgery.
3. Late arrhythmias - Like sinus node dysfunction, including sinus bradycardia, sinus pauses,
and chronotropic impairment in most of the patients 10 years postoperatively; however,
significant atrial and ventricular arrhythmias appear to be uncommon.
Suggested Reading :
1. Craig JM ,Darling RC, Pothney WB, Total anomalous pulmonary venous drainage on right
side of heart; report of 17 autopsied cases not associated with other major cardiovascular
anomalies. Lab invest 1957; 6 (1) 44-64.
2. Bando K. . Turentine MW., Ensing GJ, Surgical management of total anomalous pulmonary
venous drainage – 30 year trend. Circulation, 1996,94 (9th suppliment) II12-6
3. Tanel RE., Kirshbom PM, Paridon SM , Hartman DM et al. Long term non invasive
arrhythmia assessment after total anomalous pulmonary venous connection repair. Am Heart J.
2007: 153 (2):267-74
4. Cooley DA, Oscar VC, MD, and Fernando MP, Repair of Total Anomalous Pulmonary Venous
Return: Results after 47 Years. Tex Heart Inst J. 2008; 35(4): 451–453.