Abstract
Primary pulmonary vein stenosis (PPVS) is a rare congenital cardiac anomaly. The surgical management of PPVS requires anatomical delineation of pulmonary venous drainage along with exact localization of the stenosis. Since echocardiography cannot visualize the whole length of pulmonary veins (PV), computed tomography (CT) pulmonary venography becomes necessary. Three-dimensional (3D) reconstruction helps in planning PV augmentation and for prognostication. Here, we present 3D reconstructed CT pulmonary venography images of a 3-month-old child who presented to us with PPVS.
Keywords: Pulmonary vein stenosis, Cardiac anomaly, 3D CT
Introduction
Primary pulmonary vein stenosis (PPVS) is a rare congenital cardiac anomaly which is infrequently reported [1]. It usually presents in neonates and infants. Clinical manifestation ranges from cyanosis, rapid breathing to heart failure. The treatment of PPVS is surgical with correction of the stenosis. It requires anatomical delineation of pulmonary vein (PV) drainage along with exact localization of the stenosis [2]. Echocardiography can identify turbulence at the ostia of PV opening into the left atrium (LA), but it cannot define the extent of stenosis. In such situations, three-dimensional computed tomography (3D CT) pulmonary venography becomes helpful. It aids in planning PV augmentation and for prognostication.
Here, we present 3D reconstructed CT pulmonary venography images of a 3-month-old child who presented to us with PPVS.
Case report
A 3-month-old child presented to our hospital with rapid breathing and intermittent cyanosis. On evaluation, the child was found to have osteal stenosis of PV. Figure 1 shows preoperative chest X-ray with features of pulmonary venous hypertension.
Fig. 1.
Chest X-ray showing features of pulmonary venous hypertension
CT angiography showed discrete stenosis in the ostium of the right superior pulmonary vein (RSPV) and the left pulmonary vein (LPV) (Fig. 2). LPV was formed by confluence of the left superior pulmonary vein (LSPV) and the left inferior pulmonary vein (LIPV) before it joined into LA (Fig. 3). The right inferior pulmonary vein (RIPV) ostium appeared normal (Fig. 4).
Fig. 2.
Posterior view showing stenosis in the RSPV and the common LPV
Fig. 3.
Osteal stenosis of the common LPV
Fig. 4.
Osteal stenosis of the RSPV and normal RIPV
The child also had left superior vena cava (LSVC or Lt SVC) draining into the coronary sinus (CS). The right superior vena cava (RSVC or Rt SVC) and the inferior vena cava (IVC) were normal.
Pulmonary arterial system comprising of the main pulmonary artery (MPA), right pulmonary artery (RPA), and left pulmonary artery (LPA), and its branches appeared normal.
Since there was discrete stenosis in RSPV and LPV ostium, the child was planned for augmentation of PV ostia with autologous pericardium and parents were prognosticated.
Discussion
PPVS is a rare clinical entity with presentation similar to obstructed total anomalous pulmonary venous connection (TAPVC). There is usually an intimal shelf at the level of the PV ostium. The surgical correction of PPVS requires excision of the intimal shelf and anastomosis of normal segment of PV into LA. In cases where there is long segment stenosis or difficulty in mobilizing PV, only excision of intimal shelf with augmentation of PV ostia by using pericardial patch can be performed. To prevent restenosis, the Coles procedure (sutureless technique) is presently being used [3]. In this procedure, after opening PV, anastomosis is performed between LA and pericardium around the PV using fine absorbable suture. The purpose is to prevent trauma to the PV, thereby reducing the stimulus for regrowth of obstructive tissue.
The prognosis of PPVS remains guarded with 5-year survival and reoperation-free rate of 50% [4]. These children may develop secondary PV stenosis as the child grows and may require catheter based or surgical reintervention. If left untreated, this condition is almost uniformly fatal.
Conclusion
PPVS is a rare congenital cardiac anomaly requiring early intervention for survival. 3D pulmonary venography helps in preoperative planning, prognostication, as well as postoperative assessment of surgical correction.
Funding
None.
Declarations
Ethics approval
Not applicable.
Consent for publication
Taken.
Conflict of interest
The authors declare no competing interests.
Footnotes
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References
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