No

Age at diagnosis

Sex

Symptoms & Signs

Echocardiography

Cardiac

Catheterization

Computed Tomography

Associated Anomalies

Outcome

1

15 mo

M

Murmur

3 PVs seen draining into LA

(Figure 1)

RUPV,RMPV – Superior aspect of RA 

RLPV – Inferior aspect of RA

LUPV,LLPV - LA

OS ASD

(10mm )

Hugely dilated RA,RV

Redirection of Rt. sided PVs by enlarging ASD + ASD closure

2

6 mo

M

Murmur

RUPV – SVC- RA junction

LUPV & LLPV - LA

SV ASD .Selective RUPV angiogram – RUPV draining into high SVC 

PAP = 34/6MMHG/ Qp:Qs= 5:1.

RUPV – High SVC

RMPV – SVC- RA junction

RLPVs- 3 lower PVs confluence into single trunk & drain at SVC- RA junction separately

LUPV,LLPV - LA

SV ASD (7mm)

Hugely dilated RA,RV

Redirection of Rt .sided PVs + ASD closure

3

9 yrs

M

Murmur  

RUPV  -  SVC-RA junction

3PVs - LA

(Figure 2)

1.Accessory RPV1 – Upper SVC

2.Accessory RPV2 – Lower SVC just above ASD

3.RUPV – SVC- RA junction

4.RLPV – Inferior LA(Rt. side )

5.LUPV – VV-Innominate vein

6.LMPV – superior LA(Lt. side)

7.LLPV – Inferior LA (Lt. side)

SV ASD (10mm )

Hugely dilated RA,RV

Redirection of Rt .PVs to LA, Ligation of Left accessory PV + ASD closure

4.

5 mo

M

Murmur

RUPV  – SVC

3PVC – LA

RUPV & RLPV – SVC

Qp : Qs = 3 :1

(Figure 3)

Small OS ASD

Dilated SVC

Hugely dilated RA,RV

Redirection of Rt, sided PVs by enlarging ASD + ASD closure

5.

11mo

F

Murmur

RUPV – RA

RUPV -RA 

Qp: Qs= 3.4:1.0

OS ASD

(10mm ) Dilated RA,RV

Redirection of RUPV to LA + ASD Closure  

6.

6 yrs

F

Murmur

LUPV & LLPV - VV - Innominate vein

LUPV,LLPV - VV - Innominate vein

OS ASD

(12mm )

Dilated RA,RV

PAPVC correction + ligation of VV + ASD closure

7.

5 mo

M

Murmur

RUPV – SVC- RA Junction

RMPV – SVC

SV ASD

(7 mm), PS, Dilated RA,RV,

Redirection of RUPV to LA + ASD closure

8.

6 yrs

M

Murmur

RUPV - SVC,

LUPV- VV – Innominate V RLPV,LLPV - LA

LUPV -  VV-Innominate vein

RUPV- SVC

RMPV,RLPV ,LLPV – LA

Qp: QS = 5:1

LUPV - VV – Innominate

RUPV- SVC

RLPV,LLPV - LA

OS ASD (8mm), Dilated RA,RV

Redirection of RUPV & LUPV  to LA + ligation of VV + ASD closure

9.

15mo

M

Murmur

RUPV,RLPV –LA

LUPV,LLPV -VV- Innominate V

(Figure 4)

3 Rt. PVS- LA,LLPV - LA upper pole on rt. side ,LUPV,LMPV - VV - Innominate V – Dilated SVC Extralobular left lung posterior lobe sequestration

Cor triatriatum

Small ASD

Dilated RA,RV

Redirection of Lt PVs to LA + ligation of VV = Cor triatriatum Repair

10.

4 yrs

M

Murmur

LUPV- VV – Innominate V-Dilated SVC

(Figure 5)

1.Rt sided Small accessory PVs through tortuous single vein to RLPV - Rt. upper pole LA 2.LMPV,LLPV - Lt upper pole LA

3.RUPV takes a long course & join with LUPV which takes a u turn around LPA - draining into VV – Innominate V - RSVC

S/P COA repair

Dilated RA,RV

Awaiting surgery

11.

12 days

F

Turner syndrome

LUPV -VV-Innominate - dilated SVC

(Figure 6)

LUPV,LLPV - VV- Innominate v - SVC

COA, Small ASD

Awaiting COA Sx

12.

50 yrs

F

Exertional breathlessness

Palpitations

Dilated RA,RV,PA with small PFO,ERVSP - 70-75mmHg,Mildly impaired RV

PAPVC of RUPV , RLPV to IVC- RA  JUNCTION. Qp: Qp= 2.4:1 PAP 54/23MMHG.PVRI - 17,On 100 % O2 - 12.5 ,PAP- 47mmHg

(Figure 7)

RUPV,RLPV join into large vein drain into RA just above diaphragm ,IVC

Small PFO

Redirection of Rt sided PVs through a baffle by enlarging PFO into LA

13

2 yrs

M

Murmur

RUPV & RLPV – SVC

SV ASD

Surgery

14

3 yrs

M

Murmur

RUPV,RLPV - IVC

PAPVC of RUPV,RLPV – IVC

Small RPA PAP= 23/6mmHg Qp: Qs 1.5:1

Scimitar syndrome

Hypoplastic right lung

Occlussion Of Large Collateral From Ao To R Lung  With Amplatz Duct Occluder Device

15.

2 yrs

F

Murmur

RUPV,RLPV to IVC

Scimitar syndrome

Hypoplastic right lung

OS ASD (18mm) 

Awaiting surgery around 3-4 yrs of age

16

10 yrs

F

Admitted with pneumonia

RUPV,RLPV  - IVC

LUPV,LLPV – LA

RUPV,RLPV- IVC

Scimitar syndrome

Hypoplastic right lung

Lost follow up

17

1 day

F

Tracheoesophageal fistula

Rt sided PVs to IVC

Scimitar variant Dextroposition, Esophaheal atresia

Died post  tracheostomy

18

At birth

M

Low saturation

RLPV- RA

Heterotaxy,

AVSD,Sub AS

AVSD repair

19

1 yr

M

Recurrent respiratory tract infections

RUPV,RLPV – IVC

RUPV,RLPV – IVC

High OS ASD

 (7mm )

Redirection of Rt PVS to LA

20

3mo

M

Admitted with Pneumonia

Abnormal venous connection  seen draining into the IVC  which is mildly obstructive with PIG = 16mmHg

4 PVs into LA

Accessory PV draining into IVC

Right pulmonary extra lobar sequestration with arterial supply from coeliac trunk 

Scimitar Variant

Dextroposition

PFO

On follow up

21

Day2

F

Low saturation

RLPV- IVC

3 PVs into LA

RLPV - IVC

Scimitar Variant

Dextroposition

On follow up