Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Home Print this page Email this page
Users Online:: 735

 Table of Contents  
Year : 2016  |  Volume : 19  |  Issue : 3  |  Page : 164-167

Total anomalous pulmonary venous connection in a 9-year-old girl at Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Department of Pediatrics, Pediatric Cardiology Unit, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Date of Web Publication14-Oct-2016

Correspondence Address:
Usman Muhammad Sani
Pediatric Cardiology Unit, Department of Pediatrics, Usmanu Danfodiyo University Teaching Hospital, Sokoto
Login to access the Email id

DOI: 10.4103/1118-8561.192400

Rights and Permissions

Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease in which all the four pulmonary veins drain into the right atrium instead of the left. Without surgical intervention, 80% of the patients die before the age of 1 year. We report a 9-year-old girl with unrepaired supracardiac TAPVC complicated by severe pulmonary artery hypertension. The patient was managed conservatively including the use of pulmonary antihypertensive (sildenafil), with significant improvement. She is currently on follow-up at our pediatric cardiology clinic. TAPVC requires surgical intervention in early infancy to prevent the onset of pulmonary hypertension, which may contraindicate surgery. High index of suspicion and improved diagnostic skill will enhance early diagnosis and enable timely intervention.

Keywords: Nigeria, Sokoto, total anomalous pulmonary venous connection

How to cite this article:
Sani UM, Omeneke IK, Waziri UM. Total anomalous pulmonary venous connection in a 9-year-old girl at Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria. Sahel Med J 2016;19:164-7

How to cite this URL:
Sani UM, Omeneke IK, Waziri UM. Total anomalous pulmonary venous connection in a 9-year-old girl at Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria. Sahel Med J [serial online] 2016 [cited 2020 Jul 8];19:164-7. Available from: http://www.smjonline.org/text.asp?2016/19/3/164/192400

  Introduction Top

Total anomalous pulmonary venous connection (TAPVC) is a cyanotic congenital heart disease (CHD) in which there is no direct connection between any of the 4 pulmonary veins and the left atrium. Instead, all the pulmonary veins connect to the right atrium (RA) or its tributaries.[1],[2] A patent foramen ovale (PFO) or an atrial septal defect is usually present to allow for blood flow into the left atrium and systemic circulation. This enables survival after birth.[1],[3]

TAPVC is a rare abnormality with an incidence of 4–6/100,000 live births [4] and accounts for 1–3% of all CHDs.[4],[5] Four types are recognized based on Darling classification including supracardiac, cardiac, infracardiac, and mixed types.[6],[7] The clinical presentation varies widely depending on the adequacy of interatrial communication and whether there is obstruction to the pulmonary venous return or not.[4],[8],[9] Obstruction commonly occurs in association with the infracardiac type (90% of cases) and is characterized by acute presentation in the neonatal period with severe cyanosis, heart failure, and shock.[4],[6],[9] In unobstructed TAPVC, cyanosis is often minimal, and patient may present insidiously with symptoms of pulmonary artery hypertension due to long-standing increased pulmonary blood flow.[6],[9]

TAPVC typically presents during the neonatal period or early infancy. Without surgical intervention, 80% of the patients die within the 1st year of life.[4] However, there are a few reports of cases that survive into adulthood without surgery.[4],[10],[11] To the best of our knowledge, in Nigeria, there are no documented reports of such late presenting cases. We describe a case of unrepaired supracardiac TAPVC with pulmonary hypertension in a 9-year-old girl. The report also highlights the diagnostic and treatment challenges of such a complex lesion in a resource-limited setting.

  Case Report Top

A 9-year-old girl presented to our hospital with history of recurrent cough, difficulty in breathing, cyanosis, and poor growth since 1st year of life. Her symptoms worsened 3 weeks prior to presentation as evidenced by deepening cyanosis and breathlessness at rest (New York Heart Association class IV). There was associated facial and leg swelling. She had received treatment at different health care centers in the past and had taken traditional medications without improvement. There was no family history of CHD. The patient is the 3rd child in a nonconsanguineous monogamous family setting. She was a primary 2 pupil, but school attendance was erratic on account of the illness.

On examination, she was in respiratory distress and had central cyanosis (SpO2 55%), grade 3 digital clubbing and bilateral pitting pedal edema. There were no obvious dysmorphic features. Cardiac evaluation revealed precordial bulge with scarification marks (made previously by a traditional healer). She had tachypnea (respiratory rate-56/min), tachycardia (heart rate-148/min), and tender hepatomegaly. There was left parasternal heave, normal first heart sound, but widely split and fixed second heart sound with loud P2. She had an ejection systolic murmur at the upper left sternal border.

Chest radiograph showed cardiomegaly with right ventricular (RV) apex and enlarged RA. There was also widening of the mediastinum, giving rise to figure of eight appearance [Figure 1]. Electrocardiography (ECG) revealed right axis deviation, right atrial enlargement, and RV hypertrophy [Figure 2].
Figure 1: Chest radiograph of the 9-year-old girl with supracardiac total anomalous pulmonary venous connection showing cardiomegaly with widened mediastinum, simulating figure of eight appearance

Click here to view
Figure 2: Electrocardiography tracings of the 9-year-old girl with the total anomalous pulmonary venous connection. Note the presence of the right axis deviation, right atrial enlargement, and right ventricular hypertrophy

Click here to view

Two-dimensional echocardiography showed dilated RA and right ventricle with small left-sided chambers. There was no demonstrable communication between the pulmonary veins and the left atrium. Instead, the pulmonary veins were seen to form a confluence, which drained through a left vertical vein and superior vena cava into RA, consistent with supracardiac TAPVC. There was associated stretched open PFO with right to left shunt and intact interventricular septum with paradoxical motion. Color flow Doppler showed severe tricuspid regurgitation (TR) and moderate pulmonary regurgitation whereas continuous wave Doppler revealed pulmonary artery hypertension (TR peak gradient of 66 mmHg). Cardiac catheterization and advanced cardiac imaging such as computed tomography (CT) pulmonary angiography could not be done for the patient due to nonavailability of such facilities in our hospital.

She was managed conservatively with intranasal oxygen, anti-heart failure medications (diuretics and digoxin), and pulmonary antihypertensive (sildenafil). Her clinical condition gradually improved as evidenced by a resolution of heart failure symptoms and improvement in pulse oxygen saturation, which rose from 55% to 80%. She was subsequently discharged after a prolonged hospital stay (4 weeks). However, she was readmitted 3 months after discharge due to recurrence of heart failure with bronchopneumonia. She was again discharged following successful medical treatment and is currently on follow-up at the pediatric cardiology clinic. Her clinical condition had remained fairly stable during the last 8 months of follow-up.

  Discussion Top

This report highlights an unusual case of TAPVC in our center. This complex lesion usually presents before infancy, but a few cases may present at a later age as demonstrated by our patient who survived up to the age of 9 years without surgical intervention. Nigro et al. reported supracardiac TAPVC in two adult patients who later had successful repair [11] while Melki et al. described a rare case in a 61-year-old patient.[12]

The hemodynamic factors that favor prolonged survival in unrepaired TAPVC include large interatrial communication and the absence of obstruction to pulmonary venous flow.[4] These factors enable adequate blood mixing, better systemic blood flow, and tissue oxygenation. Though the index patient had only a small interatrial communication in the form of stretched open PFO, she had no evidence of pulmonary venous obstruction, which may account for her relative longevity. The patient had supracardiac TAPVC which, unlike infra cardiac type, is less commonly associated with venous obstruction and more likely to survive longer.[1],[6]

The diagnosis of supracardiac TAPVC in the patient was based on characteristic clinical and investigation findings. History of recurrent chest infection and heart failure in the presence of cyanosis suggest cyanotic lesion with increased pulmonary blood flow. The additional findings of widely split S2, left parasternal heave, figure of eight radiographic appearance, and right atrial and RV enlargement on ECG are compelling evidence of supracardiac TAPVC in the patient.[4],[6] Not surprisingly, the diagnosis was confirmed by subsequent echocardiographic evaluation. Additional imaging modalities such as magnetic resonance imaging and CT angiography with three-dimensional reconstruction, which might enable better delineation of venous pathways and site of drainage, could not be done for the patient as these facilities were not available. This is a major limitation since the accurate description of venous channels is necessary before surgery.[4]

Early surgical intervention in TAPVC is important before the onset of obstructive pulmonary artery hypertension, which may contraindicate surgery.[4],[7] Pulmonary hypertension may occur when there is an obstruction to pulmonary venous flow (obstructed TAPVC) or secondary to long-standing increased pulmonary flow in patients with unobstructed TAPVC.[7] Our patient had echocardiographic evidence of severe pulmonary artery hypertension at presentation. Though there have been reports of children with TAPVC undergoing surgery in adolescence or even adulthood,[8],[11] we do not have facilities for cardiac catheterization to establish with certainty whether our patient is still operable. Unfortunately, the parents could not afford referral abroad for further evaluation and management. She was nevertheless stabilized on medical treatment including the use of sildenafil, a pulmonary vasodilator with antihypertensive property.

Our report highlights some of the challenges that continue to hinder optimum patient's care in many developing nations including Nigeria. These include late presentation/referral, poverty, lack of facilities and skill for early diagnosis, and limited access to efficient and affordable cardiac care services.[13],[14] These problems are also responsible for the higher morbidity and mortality of CHD among children in many developing countries of Africa compared to those in the Western world.[13] Though our patient was correctly diagnosed and had remained fairly stable on medical treatment, the prognosis is predictably ominous. This is because obstructive pulmonary vascular disease will ultimately develop unless surgical intervention is offered quickly. We, therefore, recommend the establishment of functional cardiac centers across the country. This will go a long way in making the outcome of CHD less gloomy for our patients.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Keane JF, Fyler DC. Total anomalous pulmonary venous return. In: Keane JF, Lock JE, Fyler DC, editors. Nadas Pediatric Cardiology. 2nd ed. Philadelphia: Saunders Elsevier; 2006. p. 773-81.  Back to cited text no. 1
Bernstein D. Congenital heart disease. In: Behrman RE, Kliegman RM, Jensen HB, editors. Nelson Textbook of Paediatrics. 17th ed. Philadelphia: Saunders Elsevier; 2004. p. 1538-40.  Back to cited text no. 2
Reardon MJ, Cooley DA, Kubrusly L, Ott DA, Johnson W, Kay GL, et al. Total anomalous pulmonary venous return: Report of 201 patients treated surgically. Tex Heart Inst J 1985;12:131-41.  Back to cited text no. 3
Gupta CD, Satpathy M. Total anomalous pulmonary venous connections. In: Sathpathy M, Mishra BR, editors. Clinical Recognition of Congenital Heart Diseases. New Delhi: Jaypee Brothers Medical Publishers; 2008. p. 317-24.  Back to cited text no. 4
Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002;39:1890-900.  Back to cited text no. 5
Park MK. Total anomalous venous return. In: Pediatric Cardiology for Practitioners. 5th ed. Philadelphia: Elsevier/Mosby Inc.; 2007. p. 249-54.  Back to cited text no. 6
Geva T, van Praagh S. Anomalies of the pulmonary veins. In: Allen HD, Shaddy RE, Driscoll DJ, Feltes TF, editors. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 7th ed. Philadelphia: Lippincott Williams and Wilkins; 2008. p. 776-86.  Back to cited text no. 7
Neirotti RA, Alvarez CE, Campos MD, Quaglio MT, Vazquez JC. Total anomalous pulmonary venous connection. Scand J Thorac Cardiovasc Surg 1991;25:97-100.  Back to cited text no. 8
Wang PY, Hwang BT, Lu JH, Lee PC, Tiu CM, Weng ZC, et al. Significance of pulmonary venous obstruction in total anomalous pulmonary venous return. J Chin Med Assoc 2004;67:331-5.  Back to cited text no. 9
Naha K, Vivek G, Shetty RK, Nayak K. Late presentation of TAPVC with multiple cerebral abscesses. BMJ Case Rep 2013;2013. pii: Bcr2013009778.  Back to cited text no. 10
Nigro JJ, Choi S, Graziano J, Gandy KL, Guerrero-Tiro LM, Cleveland DC. Modified superior repair for supracardiac total anomalous pulmonary venous connection in the adult. Ann Thorac Surg 2007;84:312-3.  Back to cited text no. 11
Melki J, Kovarsky S, Redonnet M, Arrignon J, Bessou JP, Mouton-Schliefer D, et al. Total anomalous pulmonary venous return in a 61 year-old adult. Ann Chir 1992;46:722-4.  Back to cited text no. 12
Mocumbi AO. The challenges of cardiac surgery for African children. Cardiovasc J Afr 2012;23:165-7.  Back to cited text no. 13
Zühlke L, Mirabel M, Marijon E. Congenital heart disease and rheumatic heart disease in Africa: Recent advances and current priorities. Heart 2013;99:1554-61.  Back to cited text no. 14


  [Figure 1], [Figure 2]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
  Case Report
   Article Figures

 Article Access Statistics
    PDF Downloaded113    
    Comments [Add]    

Recommend this journal