Partial anomalous pulmonary venous connection is a congenital
abnormality that affects less than 1% of the population. Treatment of this
malformation is usually surgical, and it has an excellent result. However, the
presence of another vascular abnormality, for example, a persistent left
superior vena cava, may be a complicating factor to corrective surgeries. In
this case, we report an unusual evolution of a patient submitted to surgical
correction of partial anomalous pulmonary venous connection. This patient
presented with severe pulmonary hypertension and left to right shunt due to
reverse flow in a persistent left superior vena cava, which was not diagnosed
during the previous cardiac surgeries.
Keywords: Congenital Heart Disease;
Pulmonary Hypertension; Pulmonary Veins
pulmonary venous connection (PAPVC) is a congenital abnormality characterized by fusion failure of the embryologic
pulmonary venous system with the left atrium. This entity is a rare congenital
anomaly described in 0.4 to 0.7% of autopsies . The definitive management of PAPVC consists in
surgical separation of the pulmonary venous system from the systemic venous system,
through redirection of an aberrant pulmonary venous drain into the left atrium . During surgical
repair of this pathology, about 16% of the patients present another congenital
vascular anomaly, for example, a persistent left superior vena cava (PLSVC) . PLSVC is the
most common congenital venous anomaly, with an incidence of approximately 0.3%
to 0.5% in the general population, and up to 10% in patients with other
concomitant congenital heart diseases .This case aims to report an unusual evolution
after PAPVC surgical correction in a patient who presented concomitantly PLSVC.
Patient Characterization and Description of
The Technique Employed
female patient presented to a cardiac surgery outpatient clinic with
progressive dyspnea for about 5 years and no other complaints. At physical
examination, there was a systolic heart murmur 3+/6+ at the left lower sternal
border and apex, and no other relevant findings. This patient had already
undergone two previous cardiac surgeries. First when she was 6 years-old
(medical report summary indicating an interatrial communication closure), and
the second at 39 years old, when she presented a right lower pulmonary blood
flow drainage into the coronary sinus. The surgery was performed by unroofing
the coronary sinus into the left atrium and occluding the coronary sinus ostium
in the right atrium, in order to redirect the right pulmonary blood flow to the
left atrium. Soon after discharge of the second surgery, the patient had
syncope due to an atrioventricular block and was treated with a permanent
At the time of the
current outpatient evaluation, she presented a transesophageal echocardiogram
(TEE) demonstrating right heart overload (right ventricle diameter = 4.9cm), pulmonary
hypertension (trans-tricuspid gradient = 73 mmHg) and left-to-right shunt
(Qp/Qs = 2.2). The anatomic source of shunting was not readily identified, even
with agitated saline injection in a peripheral vein. A dilated coronary sinus
and PLSVC were also observed during TEE.
angiotomography demonstrated right heart and common pulmonary artery dilatation
(diameter = 36 mm), compatible with pulmonary hypertension. It also
demonstrated interventricular septum rectification, and disclosed a PLSVC,
receiving flow from the hemiazygous vein and draining into the coronary sinus (Figure 1). In this exam was possible to detect signs of elevated coronary sinus pressure
and hemiazygous vein dilatation. It was also noted collateral vessels draining
from LSVC to right superior vena cava (Video 1). Magnetic resonance
imaging was not performed since the patient had a non-conditional pacemaker.
catheterization was performed and evidentiated a mean pulmonary arterial
pressure of the 37 mmHg and a left to right shunt (Qp/Qs = 2.3). Venography
performed through contrast injection via the basilic vein disclosed a
persistent LSVC connecting to the coronary sinus, observing a reverse flow from
the coronary sinus to the LSVC and hemiazygos vein (Figure 2). Oximetry was
collected from the lower portion of the LSVC and demonstrated 97.7% oxygen
saturation, confirming the presence of a shunt with retrograde flow in the
LSVC. Considering the high surgical risk and the adequate symptomatic response
to sildenafil, it was opted to maintain the patient in pharmacological
management. This case demonstrates an unusual evolution after a PAPVC surgical correction,
attributed to the presence of another concomitant congenital vascular
malformation not identified during the pre-operative investigation.
PAPVC is a congenital abnormality characterized by fusion
failure of the embryologic pulmonary venous system with the left atrium . This results in
one to three pulmonary veins draining to structures other than the left atrium. Considering the
rule of proximity in embryology: right pulmonary veins usually drain to vena
cava or right atrium; while left pulmonary veins drain to coronary sinus, left
superior vena cava or innominate vein . Other
congenital vascular abnormality is the PLSVC. The development of major systemic
veins results from growth and involutions of different venous drainages systems
during fetal development . The closure failure
of the left anterior cardinal vein will result in a PLSVC, which usually drains
the left subclavian and jugular veins into the right atrium via an enlarged
coronary sinus . Data about the concomitance
of these two abnormalities are scarce and the true incidence of this
association remains unknown. However, a case series of patients submitted to
surgical correction of PAPVC showed that 15.7% of them presented a PLSVC .
In the reported case the
patient was submitted to a PAPVC surgical correction according to the
traditional technique, in which pulmonary veins are routed to the left atrium
by unroofing the coronary sinus, and the ostium of the coronary sinus is closed
. However, this correction was prejudiced by
the misdiagnosis of a PLSVC during pre and intraoperative moments. The
misdiagnosis, although uncommon and avoidable, can occur if the presence of a
PLSVC is not considered in the case. Data from a selected pediatric population
submitted to cardiac surgeries for congenital heart disease revealed that in
the preoperative period almost 20% of the PLSVC diagnosis could be missed by
echocardiography-only investigation , which
is the first-line imaging tool for PLSVC diagnosis .
Lu S. et al. comment, for example, a case of partial anomalous pulmonary venous
connection misdiagnosed as primary pulmonary arterial hypertension because the
two high right superior pulmonary veins drained into the superior vena cava and
limited the shunt of patent foramen ovale .
Abnormal venous connections can
be identified by computed tomography or magnetic cardiac resonance or, even so,
intraoperatively. The identification of PLSVC during surgery is made by the
dissection of the thymic fat and visualization of the left innominate vein. However,
in adult patients referred to surgery, this abnormality may be missed if not
carefully and properly researched. The presence of a PLSVC may be a benign
finding with no clinical repercussions in patients with no other anomaly of the
anatomy, but, in those patients that with unroofed coronary sinus, it can be
associated with arterial desaturation and paradoxical systemic embolism . In the presented case, due to surgical correction
of PAPVC, our patient developed an anatomy similar to those patients who
congenitally present an unroofed coronary sinus. Besides, the surgery performed
lead the drainage of the PLSVC to the left atrium, which traditionally cause a
right-to-left shunt . Nevertheless, in our
patient a left-to-right shunt was disclosed during investigations. This fact,
as in the case reported by Chen, et al. ,
occurred because of an elevated left atrium pressure, which resulted in a
reverse flow (caudal-to-cranial) on the PLSVC. A similar case, however in an
infant, was reported by Quarti A. et al. In this description, disconnected left superior vena cava draining into
the left atrium through the left appendage,
associated with atrial septal defect and right pulmonary veins anomalous connection was successfully corrected by
rerouting the pulmonary venous return into the left atrium and transposing the
left vena cava on the right appendage .
this case we report an unusual evolution after a PAPVC surgical correction in a
patient presenting severe pulmonary hypertension and a left-to-right shunt,
which occurred due to the presence of a concomitant vascular anomaly, which was
not diagnosed neither in the preoperative period, nor in the intraoperative
Figure 1: (A): Contrast-enhanced computed
tomography coronal image demonstrating the presence of a persistent left
superior vena cava. (B): Diversion
of right pulmonary drainage to the left atrium through the coronary sinus
cineangiography demonstrating the presence of a persistent left superior vena cava.
(B): The drainage of the left
superior vena cava into the coronary sinus.
1. Majdalany DS, Phillips SD, Dearani JA, Connolly HM, Warnes CA (2010) Isolated partial anomalous pulmonary venous connections in adults: twenty-year experience. Congenit Heart Dis 5: 537-545.
2. Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, et al. (2007) Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection. Ann Thorac Surg 84: 2020-2026.
3. Clarke NS, Murthy RA, Guleserian KJ (2017) Persistent Left Superior Vena Cava: Incidence and Management in Patients Undergoing Repair of Partial Anomalous Pulmonary Venous Connection. World J Pediatr Congenit Heart Surg 8: 440-444.
4. Ho CL, Tsai IC, Lin MC, Jan SL, Fu YC (2014) Anomalous connection of the right pulmonary vein to the coronary sinus in a young infant. Pediatr Neonatol 55: 407-409.
5. Giuliani-Poncini C, Perez MH, Cotting J, Hurni M, Sekarski N, et al. (2014) Persistent left superior vena cava in cardiac congenital surgery. Pediatr Cardiol 35: 71-76.
6. Irwin RB, Greaves M, Schmitt M (2012) Left superior vena cava: revisited. Eur Heart J Cardiovasc Imaging 13: 284-291.
7. Van Praagh R, Harken AH, Delisle G, Ando M, Gross RE (1972) Total anomalous pulmonary venous drainage to the coronary sinus. A revised procedure for its correction. J Thorac Cardiovasc Surg 64: 132-135.
8. Lentini S, Recupero A (2011) Recognition of persistent left superior vena cava in non-congenital patients undergoing cardiac surgery. Perfusion 26: 347-350.
9. Lu S, Wei L, Zheng J, Wang C (2017) Atypical Case of Partial Anomalous Pulmonary Venous Connection Misdiagnosed as Primary Pulmonary Arterial Hypertension. Heart Surg Forum 20: E231-E233.
10. Chen YA, Joshi SB, Crean AM, Yan AT, Latter DA, et al. (2011) Persistent left superior vena cava with retrograde drainage from the left atrium into the left brachiocephalic vein. J Am Coll Cardiol 58: 2141.
11. Quarti A, Di Eusanio M, Pierri MD, Di Eusanio G (2005) Left superior vena cava draining into the left atrium, associated with partial anomalouspulmonary venous connection: surgical correction. J Card Surg 20: 353-355.
Citation: da Silveira LMV, Tagliari AP, Matte BS, Kochi AN, Foppa M (2018) Pulmonary Hypertension Secondary to Reverse Flow in Left Superior Vena Cava Presenting as Late Evolution of a Pulmonary Anomalous Partial Venous Communication Surgical Correction. J Surg: JSUR-1182. DOI: 10.29011/2575-9760. 001182