QUAD #18: Vascular Rings & Aortic Uncrossing with Dr. Carl Backer

There are four types of vascular rings: double aortic arch, right aortic arch with left ligamentum arteriosum and aberrant left subclavian artery, right aortic arch with left ligamentum arteriosum, and left aortic arch with right-sided descending aorta and right ligamentum arteriosum.

Many surgeons are starting to approach some vascular ring revisions thoracoscopically, specifically the right arch with aberrant left subclavian and double arches with an atretic left subclavian.

Robert Gross was the first surgeon to describe vascular rings and coined the phrase 'vascular ring'.

Robert Gross performed the first vascular ring division in 1945 using simple ligatures to divide both the ligamentum and the arch.

Willis Potts developed a safer technique by suturing the stumps of the divided arch, which is now the standard of care.

Dr. Potts was the first to describe surgical repair of a pulmonary artery sling in 1954.

The Society of Thoracic Surgeons developed a classification scheme for vascular rings in 2000 including double aortic arch, right aortic arch with left ligamentum, innominate artery compression, and pulmonary artery sling.

Children with double aortic arch present quite early within the first month of life, while right aortic arch tends to present later.

Most commonly, patients with vascular rings present with stridor or recurrent upper respiratory tract infections.

Dr. Backer still finds barium swallow helpful in marginal cases, though the diagnostic schema has changed.

In a 2005 paper, computed tomography became the method of choice for vascular ring diagnosis and really replaced the barium swallow.

Preoperative bronchoscopy is strongly recommended, often done at the time of vascular ring division.

Preoperative echocardiogram is strongly recommended because probably 10 to 15% of vascular ring patients will have a cardiac diagnosis.

For double aortic arch, the right arch is commonly dominant, a few will have a dominant left arch, and only about 5% of arches will be balanced.

Repair of double aortic arch includes a small muscle sparing left anterior lateral thoracotomy using vascular clamps to over sew the stumps.

Left dominant double aortic arches require a different approach through a right thoracotomy.

Dr. Potts originally performed pulmonary artery sling repair through a right thoracotomy, which is a very difficult approach.

Pulmonary artery sling cases are now mostly completed through median sternotomy with cardiopulmonary bypass.

In pulmonary artery sling, the pulmonary arteries are typically smaller than normal because they've been compressed in utero.

Right sided aortic arches can be associated with a Kommerell diverticulum.

Some patients with right aortic arch require reoperation when only a simple ligamentum division has been performed and later have recurrence of symptoms from a large Kommerell diverticulum.

Primary resection of the Kommerell diverticulum includes transferring the left subclavian artery to the left carotid artery to allow the space around the esophagus and trachea to be widely open without compression.

Fetal diagnosis has become a game changer for vascular ring management and operative timelines.

The median age at vascular ring repair dropped from 24 months to 13 months if there was a prenatal diagnosis.

The mean weight at the time of vascular ring surgery dropped from almost 20 kilos to about 10 kilos with fetal diagnosis.

Patients with fetal diagnosis of vascular rings had fewer respiratory tract infections because surgeons were operating on them sooner before they got sick.

There was no difference in post-operative complications between fetal diagnosis and non-fetal diagnosis groups for vascular ring repair.

The length of stay for fetal diagnosis patients was shorter than for other vascular ring patients.