Valve-sparing aortic root replacement in children: Outcomes from 100 consecutive cases

Intraoperative echocardiographic coronary artery imaging in congenital and acquired heart disease

“…Connective tissue disorders, such as Marfan Syndrome or Loeys-Dietz syndrome, or primary aortic valve abnormalities may lead to significant aortic root dilation and require valve-sparing aortic root replacement with concomitant coronary artery re-anastomosis. 6 Acquired heart disease such as Kawasaki disease can lead to coronary artery aneurysm formation, stenosis, and/or occlusion, and coronary artery bypass grafting may be necessary to maintain distal coronary perfusion. 7 Current guidelines that outline the clinical indications and optimal imaging views for transoesophageal echocardiography 2 do not explain intraoperative coronary artery anatomy imaging.…”

Valve-Sparing Operations: What We Know and What We Don’t Know

“…Two articles recently published might be of interest to the readership, summarizing the lessons that we have learned with now well over 100 consecutive cases of VSARRs performed in pediatric patients over two decades. 2,3 Our case series has a large prevalence of patients with structurally normal hearts in the setting of connective tissue disorders but also includes patients with conotruncal anomalies and failed Ross procedures. As congenital heart surgeons, we are now also seeing a modest increase in the number of patients who have undergone three-stage palliation of hypoplastic left heart syndrome and present with competent or mildly regurgitant pulmonary (neoaortic) valves and aneurysmal enlargement of the proximal aortopulmonary amalgamation.…”

“…As far as maximal root diameter is concerned, the indications we currently utilize for surgical intervention have been reported elsewhere. 2 In pediatric patients with Marfan syndrome (MFS), we currently use criteria that have been put forward for adult patients, given the rarity of rupture before teen-age years even at sizeable diameters and very high Z -scores. Criteria for intervention are more elusive for patients with Loeys-Dietz syndrome (LDS).…”

Pediatric valve-sparing aortic root replacement: Approaching perfection

“…Concerns over the development of aortic valve insufficiency or a pseudoaneurysm necessitating further surgery have been the primary arguments against these operations. 1 In this issue of the Journal, Fraser and colleagues, 2 present single-institution data from a 20-year period describing 100 consecutive cases of valve-sparing aortic root replacement (VSRR) in patients aged less than 18 years (median, 13.6 years). As expected, the majority of the patients had underlying connective tissue disease, such as Marfan syndrome (51%) or Loeys-Dietz syndrome (39%).…”

“…Fraser and colleagues 2 show that the reimplantation VSRR is an effective, reproducible technique in children. With midterm follow-up, the risk for surgical reintervention is low.…”