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Disclosure: Authors have nothing to disclose with regard to commercialsupport.Received 21 April 2018; revised 21 May 2018; accepted 2 June 2018.

⇑ Corresponding author at: Pediatric Cardiology Department, ChestDisease Hospital, Ministry of Health, P.O. Box 4081, Safat 13041, Kuwait.

E-mail address: jarrah5@hotmail.com (M. Al Qbandi).

Transcatheter therapy of partial anomalouspulmonary venous connection with dualdrainage and coarctation of the aorta ina single patient

1016-7315� 2018 Production and hosting by Elsevier B.V. on behalf of King Saud University. This is anopen access article under theCCBY-NC-N

(http://creativecommons.org/licenses/by-nc-nd/4.0/).

Peer review under responsibility of King Saud University.

URL: www.ksu.edu.sa

https://doi.org/10.1016/j.jsha.2018.06.003Production and hosting by Elsevier

Mustafa Al Qbandi a,⇑, Mariappa Thinakar Vel a

a Pediatric Cardiology Department, Chest Disease Hospital, Ministry of Health

aKuwait

A 12-year-old boy was found to have aortic coarctation and a partial anomalous pulmonary venous connection.Historically, multiple cardiac pathologies, such as in the present case, required a surgical approach. We describetranscatheter treatment of the coarctation with a stent and occlusion of the partial anomalous pulmonary venousconnection with an Amplatzer vascular plug in a single patient without complications.

� 2018 Production and hosting by Elsevier B.V. on behalf of King Saud University. This is an open access articleunder the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Keywords: Coarctation of aorta, Dual drainage, Partial anomalous pulmonary venous connection, Stent, Vascularplug, Vertical vein

1. Introduction

Partial anomalous pulmonary venous connec-

tion (PAPVC) is an extremely rare congenitalcondition where one or more of the pulmonaryveins are connected to the venous circulation. Itsprevalence within the general population is0.4–0.7% [1]. Approximately 90% of all PAPVCsoriginate from the right lung, 7% originate fromthe left lung, and 3% of patients are found to havebilateral PAPVCs originating from both lungs

connecting to the superior vena cava (SVC), theinferior vena cava (IVC), the right atrium, or theinnominate vein. Partial anomalous pulmonaryvenous connections are frequently associated withatrial septal defects [1–3], and are rarely associatedwith other congenital abnormalities of the heart[4]. Echocardiography is the initial modality ofchoice for the noninvasive detection of PAPVC[1]. Generally, patients with a partial anomalouspulmonary venous connection, if symptomatic orshowing evidence of significant left-to-rightshunting, are treated with surgery. Occasionally,

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312 AL QBANDI, THINAKAR VELTRANSCATHETER THERAPY OF PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION

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it is possible to treat partial anomalous pulmonaryvenous connections percutaneously by means ofan Amplatzer occlusion device (AGA MedicalCorporation, Plymouth, USA), although this isonly feasible when the anomalous pulmonaryveins connect both to the left atrium and the sys-temic veins. To our knowledge, only few suchcases have been previously described in the liter-ature [5–7]. We report a case of transcatheter treat-ment of coarctation of the aorta and a partialanomalous pulmonary venous connection in a sin-gle patient.

2. Case report

A 12-year-old boy presented with persistentheadache and leg pain for the past 8 months. Onexamination, he was found to be hypertensivewith blood pressure 132/90 and had an ejectionsystolic murmur. Subsequent echocardiogramrevealed evidence of an aortic coarctation beyondthe origin of the left subclavian artery and partialanomalous drainage of the left upper lobe pul-monary vein via a vertical vein into the systemicleft innominate vein. He underwent cardiacmagnetic resonance imaging, which showedcoarctation of aorta and left upper pulmonary veindraining to left innominate vein. The patient’smagnetic resonance angiogram study of brainshowed no cerebral artery aneurysms. The patientunderwent cardiac catheterization and angiogra-phy revealed left aortic arch with normal branch-ing and a coarctation just at the origin of the leftsubclavian artery (Fig. 1) with a peak-to-peak

Figure 1. Aortic angiogram illu

gradient of 20 mmHg and a minimum coarctationdiameter of 7 mm and poststenotic diameter of 20mm and the diameter of descending aorta at thelevel of diaphragm was 16 mm. A 3.4-cm BareCheatham Platinum stent (NuMed Inc., NewyorkCity, USA) mounted on a 20-mm Z-med II balloon(NuMed Inc., Newyork City, USA) was positionedacross the coarctation and deployed (Fig. 2), withno residual gradient after stenting.Innominate vein angiogram showed no left

superior vena cava but a significant left-to-rightshunt via the anomalous venous connection fromthe left upper lobe pulmonary vein via a verticalvein into the systemic left innominate vein. Theoxygen saturations in the left and right pulmonaryvein were 98%, in the left innominate vein 98%,and in the right innominate vein 82%. A repeatprocedure was undertaken for occlusion of thepartial anomalous pulmonary venous connection.Angiography of the left pulmonary artery showedgood size left pulmonary artery and confirmeddrainage of the left upper pulmonary vein via avertical vein into the innominate vein (Fig. 3).Angiography of the right pulmonary arteryshowed good size right pulmonary artery and allright sided pulmonary veins draining to leftatrium and no atrial septal defect. The use of 6FBerman wedge catheter balloon occlusion veno-gram of the vertical vein showed dual supply ofthe left upper pulmonary vein, one to a verticalvein and the other to the left lower pulmonaryvein which is draining to the left atrium (Fig. 4).While occluding the vertical vein, the innominatevein pressure remained at 8–10 mmHg. A

strating aortic coarctation.

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Figure 2. Aortic coarctation after treatment with a 3.4-cm Bare CP stent (NuMed Inc., Newyork City, USA). CP = Cheatham platinum.

Figure 3. Balloon occlusion venogram of the vertical vein showed dual supply of left upper pulmonary vein showing dual drainage of the leftupper pulmonary vein via a vertical vein and the left atrium (arrow).

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AL QBANDI, THINAKAR VEL 313TRANSCATHETER THERAPY OF PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION

16 � 12-mm Amplatzer Vascular Plug II (AGAMedical Corporation, Plymouth, USA) was usedto occlude the vertical vein before its junction withthe innominate vein (Fig. 5). Repeat angiographyconfirmed no residual leak. The patient remainedstable and a chest X-ray revealed that both pros-theses remained well seated.

3. Discussion

A partial anomalous pulmonary venous connec-tion occurs when at least one of the pulmonaryveins connect to the right atrium directly or indi-

rectly through one or more of its venous tribu-taries [8]. No definitive classification system hasbeen proposed for this disorder, although Alsoufiet al. [9] have proposed five subtypes of partialanomalous pulmonary venous connection, whichinclude the following: (1) right partial anomalouspulmonary venous connection to the superiorcaval vein: the most common type, in which theanomalous pulmonary veins attach to the lowerside of the superior caval vein or the superiorcaval vein/right atrium junction; (2) right partialanomalous pulmonary venous connection to theright atrium. The right pulmonary veins connect

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Figure 4. Diagram showing partial anomalous pulmonary venousconnection with dual drainage and coarctation of the aorta.

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directly to the right atrium; (3) right partialanomalous pulmonary venous connection to theinferior caval vein (scimitar syndrome). Theanomalous right pulmonary vein, generallydraining the entire right lung, descends in acephalocaudal direction toward the diaphragm,then joins the inferior caval vein or inferior cavalvein/right atrial junction; (4) left partial anoma-lous pulmonary venous connection. The leftpulmonary veins connect to the left innominate

Figure 5. Amplatzer Vascular Plug II (AGA Medical Corporation, Plymouthe innominate vein.

vein by way of an anomalous vertical vein; and(5) bilateral partial anomalous pulmonary venousconnection; a rare form of partial anomalous pul-monary venous connection. Most commonly, theatrial septum is intact, the left superior pulmonaryvein attaches to the left innominate vein by way ofan anomalous vertical vein, and the right superiorpulmonary vein attaches to the superior cavalvein/right atrial junction.Herewedescribe a less common, type 4 variety of

partial anomalous pulmonary venous connection,in which the anomalous pulmonary vein (verticalvein) ascends into the left innominate vein, in asso-ciation with coarctation of the aorta, which was dis-covered during routine assessment. Until morerecently, such cases with multiple cardiac patholo-gies would have been treated using a surgicalapproach. However, advancement and refinementof percutaneous techniques has meant that suchcases involving multiple cardiac pathologies canbe treated using interventional percutaneoustechniques, obviating the need for surgery withvery good outcome data. Endovascular stentimplantation has become the modality of choicefor the treatment of coarctation of the aorta in olderchildren and adults, and is associated with rela-tively low morbidity and mortality [10]. The dualdrainage allowed successful percutaneous closureof the vertical vein without obstruction to thepulmonary venous flow to the left atrium. To ourknowledge, there is only one reported case [7] ofcoarctation in association with partial anomalouspulmonary venous connection treated successfullywith a percutaneous approach, thereby obviatingthe need for surgery and its associated risks.In partially abnormal pulmonary venous return

with dual drainage, transcatheter therapy can be

th, USA) was used to occlude the vertical vein before its junction with

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AL QBANDI, THINAKAR VEL 315TRANSCATHETER THERAPY OF PARTIAL ANOMALOUS PULMONARY VENOUS CONNECTION

offered in most patients. This case illustrates thefeasibility of complete interventional cure of selectcases of partial anomalous pulmonary venousdrainage with dual drainage. Even in patients withmultiple cardiac pathologies, percutaneoustreatment options should be considered in closeliaison with surgical colleagues before the deci-sion for a surgical approach to treatment is made.

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[2] Hijii T, Fukushige J, Hara T. Diagnosis and management ofpartial anomalous pulmonary venous connection.Cardiology 1998;89:148–51.

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