Definition: connection of the pulmonary vein to remnants of embryologic venous circulation rather than the left atrium. This may include all four pulmonary veins (TAPVR) or only part of it (PAPVR).
Classification: (by Darling, Rothney and Craig)
Supracardiac 50% (pulmonary veins drain via innominate vein into RA)
Infracardiac 20% (pulmonary veins drain via portal vein into RA)
Intracardiac 20% (pulmonary veins drain via coronary sinus into RA)
Mixed type 10%
An ASD is always present. 1/3 of all patients have associated other defects
Incidence 8:100.000 of live birth. Prevalence 2.2% in CHD
Depends on the PBF, which is anatomically dependent on: the left to right shunt (due to abnormal pulmonary venous drainage); the obstruction of the pulmonary drainage, if present; and on the right to left shunt (ASD and PDA).
If severely obstructed pulmonary venous drainage there will be decreased PBF, leading to severely depressed CO.
Severe pulmonary oedema and PHT associated with severe right to left shunt (ASD and PDA) leads to hypoxaemia and to severely depressed CO.
In mild to moderate pulmonary venous obstruction, there is usually increased PBF with falling PVR, leading to CCF with PHT.
If there is no pulmonary venous obstruction then there will be only mildly increased PBF, mild cyanosis, and CCF develops later.
Depends on the degree of pulmonary venous obstruction and PBF.
Early presentation is usually with severe cyanosis and low CO in neonatal period in severely obstructed patients. Later presentation usually implies mild to moderate obstruction only.
Investigations will include cardiac catherisation, angiography, MRI.
Sepsis, PPHN, cor triatrium
- No or mild obstruction usually needs no special treatment .
- Moderate obstruction presenting with CCF requires anti-CCF treatment (cardiomyopathy).
- Severe obstruction will need intubation, ventilation and sedation, stabilisation of cardiac output (inotropes) and treatment of pulmonary hypertension, even ECMO. Commence Prostaglandin E1 (20 ng/kg/min) to maintain systemic perfusion, however this may decrease further the PBF and pronounce the cyanosis.
Depends on the age, presentation, and anatomy. There are various technique with ligation of the aberrant vein and reanastomosis to the left atrial appendage, or intracardiac baffle.
- Keep intubated, ventilated, and well sedated (fentanyl for any noxious stimulus) and paralysed for 24-48hrs
- Inotropes: milrinone plus dopamine or adrenaline (plus noradrenaline). Aim for MAP >40 mmHg in neonates
- Haemodynamics: SBP >60 mmHg, MAP >40 mmHg, increasing over time. CVP 8-12 mmHg, PAP< ½xSBP
- Respiratory: balanced circulation (SpO2 75-85%) in single-ventricle physiology; normoxaemia in biventricular. Normocapnea.
- Fluid restriction: 1ml/kg/hr, trophic feeds
- Most common: pulmonary hypertension – especially in post pulmonary venous obstruction patients and younger patients.
- Postoperative pulmonary venous obstruction (in the immediate postoperative period) (see chylothorax)
- Arrhythmiaa: SVT (adenosine, digoxin), JET (slow amiodarone), bradycardia (see pacing), AV Block ( see arrhythmias)
- Low urine output: start PD
Mean ICU stay: 5days.
Mortality at 30 days: up to 90% in single-ventricle TAPVR, but 5-35% in two-ventricle physiology.
Reobstruction of pulmonary venous flow possible (2.5-13%)
 Lab Invest. 1957 Jan-Feb;6(1):44-64: Craig et al: Total pulmonary venous drainage into the right side of the heart; report of 17 autopsied cases not associated with other major cardiovascular anomalies.
 Tex Heart Inst J. 1985 Jun;12(2):131-41: Reardon et al: Total anomalous pulmonary venous return: report of 201 patients treated surgically
 Ann Thorac Surg. 2005 Feb;79(2):596-606; discussion 596-606: Hancock Friesen et al: Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution.