Interventional therapy for partial anomalous pulmonary venous connection with dual drainage.

A 6-year-old boy presented with dual drainage of left upper pulmonary vein, with connection to innominate vein inaddition to its normal connection to the left atrium. Despite relief of aortic stenosis at the age of 3 years, significant left to right shunt persisted. The dual drainage allowed successful percutaneous closure of the levoatriocardinal vein without obstruction to the pulmonary venous flow to the left atrium.

CLINICAL SUMMARY

A 3-year-old boy presented with bicuspid aortic valve and severe aortic stenosis. In addition, he had anomalous drainage of the left upper pulmonary vein (LUPV) which besides draining into the left atrium (LA) joined the innominate vein through a patent levoatriocardinal vein (LACV). This dual drainage, an unusual form of partial anomalous pulmonary venous connection, led to left to right shunt despite intact atrial septum. Severe aortic stenosis and the consequent elevation of left ventricular end diastolic pressure arguably promotes the shunt and is expected to decrease after successful balloon aortic valvuloplasty. Therefore, the partial anomalous pulmonary venous connection was not addressed at the time of balloon aortic valvuloplasty. However, despite successful balloon aortic valvuloplasty, the left to right shunt persisted with progressive dilatation of innominate vein, right atrium and right ventricle during follow-up [Figure 1a].

Figure 1

(a) Chest radiograph (PA view) showing mild cardiomegaly and prominent shadow of LACV (arrows). (b) Angiogram in left anterior oblique view showing dilated innominate vein and LACV. The constriction in LACV is well above the joining of LUPV. (c) Line diagram depicting details of venous anatomy and relevant dimensions for the interventional closure of LACV. (d) Left pulmonary artery follow-through angiogram during venous phase showing well positioned device and normal flow in LUPV. (e) Echocardiogram 1 day after the procedure showing device (star) in situ and unobstructed flow in LUPV. (f) Chest radiograph (PA view) on follow-up showing a reduction in the shadow corresponding to LACV. PA: Pulmonary artery; LACV: Levoatriocardinal vein; LUPV: Left upper pulmonary vein; ADO: Amplatzer duct occluder; Ao: Aorta; LA: Left atrium; LIJV: Left internal jugular vein; LMPV: Left middle pulmonary vein; RIJV: Right internal jugular vein; RLPV: Right lower pulmonary vein; RSVC: Right superior vena cava; RUPV: Right upper pulmonary vein

The presence of normal connection to the LA, in addition to LACV, makes it an ideal candidate for interventional therapy. An interventional closure of patent LACV was, therefore, contemplated. The ratio of pulmonary to systemic blood flow was 2.5, and pulmonary artery pressure was mildly elevated. The main concern was the risk of obstruction to the LUPV egress in the LA following device placement. The vertical length and mild constriction of the vertical channel above the joining of LUPV [Figure 1b, c and Video 1] provided safe landing zone for the occluder device. The LACV was entered with 7 Fr Swan-Ganz balloon floatation catheter, through which a 0.35” Amplatz extra stiff exchange length wire was parked in the LA. A 9 Fr Mullin's sheath was then placed over the wire through which a 14/12 mm Amplatzer duct occluder (AGA Medical Corporation, Golden Valley, Minnesota, USA) was deployed. The position of the device and the status of the flow in LUPV were confirmed with angiogram [Figure 1d and Videos 2, 3] and echocardiogram [Figure 1e and Video 4]. On follow-up, the child is symptomatically better with a significant reduction in the size of innominate vein, right atrium, and right ventricle [Figure 1f].

This case illustrates the feasibility of complete interventional cure of select cases of partial anomalous pulmonary venous drainage with dual drainage.

Videos Available on: www.annalspc.com

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.