Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve disease.

OBJECTIVE: Symmetric bicuspidizing repair has been shown to be safe and effective in the short term in adults and children with unicuspid aortic valve. Outcomes of extending this technique to patients with other forms of aortic and truncal valve disease have not been reported. METHODS: We performed a retrospective review of patients who underwent the symmetric bicuspidizing repair at Boston Children's Hospital between December 2019 and June 2022 with a contemporary comparator group of patients who underwent other forms of bicuspidization. Survival, valve-related reoperation, and the development of moderate or greater aortic or truncal valve regurgitation were assessed. RESULTS: There were 23 patients who underwent symmetric bicuspidizing repair and 18 who underwent another form of bicuspidization. Preoperative aortic regurgitation was present in 87.0%. Patients who underwent symmetric bicuspidizing repair more commonly underwent suture annuloplasty (100% vs 55.6%; P = .002) and ascending aortoplasty (78.3% vs 27.8%; P = .004). There was 1 operative mortality (2.4%) in the entire cohort and 1 late mortality. Freedom from moderate aortic regurgitation was 87.5% at 21 months after symmetric bicuspidizing repair compared with 43.5% for patients who underwent other types of bicuspidization; P = .03. Freedom from valve-related reoperation was 100% in the symmetric bicuspidizing repair group compared with 64.4%; P = .02. CONCLUSIONS: The symmetric bicuspidizing repair may be safely extended to patients with various forms of congenital aortic and truncal valve disease. Longer term follow-up will be necessary to determine the comparative effectiveness of this technique compared with neocuspidization and the Ross procedure.

Rescue ross procedure and mitral valve repair on a low birth weight preterm neonate.

Although mid- and long-term outcomes after the Ross procedure for aortic valve disease have been increasingly improving over the years, this is still a rather challenging operation in neonates and small children. This is particularly true for patients with associated congenital heart defects and critical clinical conditions. Herein we describe the application of this procedure as a rescue operation in emergency circumstances in a low-birth-weight neonate with severe aortic stenosis, aortic regurgitation and mitral regurgitation after a previous aortic coartectomy.

Congenital unicuspid aortic valve repair without cusp patch augmentation.

Unicuspid aortic valves are rare congenital malformations. Surgical repair is feasible in aortic regurgitation, and in some cases of aortic stenosis. The standard surgical approach is a bicuspidization and symmetrization with pericardial patch augmentation of valve leaflets. Herein, we are describing our original technique for bicuspidization of a unicuspid aortic valve in adults without leaflet patch augmentation. We also address the surgical management of a commissural diastasis.

Congenitally Abnormal Aortic Valve Causing Coronary Obstruction and Cardiac Arrest in Infancy.

Many instances of coronary artery anomalies are documented in the literature; however, the detection and treatment of an asymmetric, large aortic cusp causing obstruction of a coronary ostium in a symptomatic infant remains unreported. We present a case of a 2-month-old infant with an enlarged right coronary cusp obstructing the left coronary ostium, requiring emergent repair by relocating the left coronary button and reconstructing the sinus of Valsalva with autologous pericardium. This procedure preserved native aortic valve function, and the child remains asymptomatic months after discharge.

Long-Term Fate of the Neoaortic Root After Neonatal Ross Operation: A Case Series.

The aim of this study is to analyze the adaptation properties of the pulmonary autograft in four infants who underwent the Ross operation before one year of life. The patients underwent serial echocardiographic assessments of the autograft diameters at short- and long-term follow-up and values were reported as the Z scores for normal aortic and pulmonary diameters. At a median follow-up time of 18.5 years (range: 18.2-19.4 years), all the patients are alive, none requiring autograft reinterventions. This series shows excellent adaptation potential of the "infant pulmonary autograph" in the long-term, during somatic growth of the patient.

[Infective Endocarditis in Right Ventricle( RV)-Pulmonary Artery( PA) Conduit Late after the Ross Procedure;Report of a Case].

Ross procedure has been found to have a lower incidence of infective endocarditis compared to other aortic replacement procedure using prosthetic valves. We report a case of 25-year-old man who underwent Ross procedure for congenital aortic stenosis and regurgitation when he was 7 years old. He presented with fever and was highly suspected of infective endocarditis. All sets of blood cultures were positive for Heamophilus parainfluenzae. Autologous pericardial pulmonary valve was severely stenotic and computed tomography (CT) scan and radio isotope (RI) scan revealed infection at the stenotic valve. We performed right ventricle (RV)-pulmonary artery (PA) conduit replacement and he was discharged after completion of intravenous antibiotic treatment. We experienced a rare case of infective endocarditis in a patient late after Ross procedure. Prophylaxis against infective endocarditis is mandatory even in patients with infection resistant Ross procedure.

Late results of the Ross procedure.

OBJECTIVE: The study objective was to examine the long-term results of the Ross procedure in a cohort of patients followed prospectively for more than 2 decades. METHODS: From 1990 to 2004, 212 consecutive patients with a median age (interquartile range) of 34 years (28-41) underwent the Ross procedure; 82% had congenital aortic valve disease. The technique of aortic root replacement was used in one half of the patients. Patients have been followed prospectively for a median (interquartile range) of 18.0 (14.6-21.2) years. Valve function was assessed by echocardiography. RESULTS: Cumulative mortality at 20 years was 10.8% (95% confidence interval, 6.5-17.8). Thirty patients required Ross-related reoperations and 3 for coronary artery disease. The cumulative probability of Ross-related reoperations at 20 years was 16.8% (95% confidence interval, 11.3-24.5), on the pulmonary autograft was 11.5% (95% confidence interval, 7.2-18.0), and on the pulmonary homograft was 8.2% (4.6-14.7). The implantation technique was not associated with the cumulative incidence of reoperations on the pulmonary autograft. The development of moderate or severe aortic insufficiency and pulmonary homograft dysfunction increased with time. At 20 years, the probability of aortic insufficiency was 13% (95% confidence interval, 8.0-20.3) and of pulmonary homograft dysfunction was 19.7% (95% confidence interval, 13.9-27.2). Preoperative aortic insufficiency was associated with increased odds of postoperative aortic insufficiency. CONCLUSIONS: The long-term results of the Ross procedure are excellent regardless of the implantation technique, but there is a progressive deterioration of function of both semilunar valves.

Double semilunar valve replacement in complex congenital heart disease using decellularized homografts.

OBJECTIVES: Patients with complex congenital heart disease often require multiple reoperations, resulting in increased rates of operative morbidity and mortality. Decellularized heart valves (DHVs) have led to reduced reoperation rates compared with current other valve substitutes when used for pulmonary valve replacement and have also shown very auspicious early results in aortic valve replacement. The aim of the work was to analyse the outcome of a single-stage decellularized valve implantation in the aortic and pulmonary position. METHODS: A prospective follow-up of all patients who received a single-stage double semilunar valve replacement using DHV at our institution. RESULTS: Since 2011, 5 patients underwent combined semilunar valve replacement with DHV at our institution: two following a Ross procedure (31-year-old man and 38-year-old woman) and 3 after repair of the truncus arteriosus communis (2-year-old boy and 11-year-old and 16-year-old girls). All patients had undergone previous surgery. The Ross patients had preceding valve procedures, and the patients with truncus arteriosus communis had undergone 1 repair and subsequent operative procedures. Despite challenging operations (median bypass time 346 min, range 275-477 min; median cross-clamp time 229 min, range 140-307 min), there was no perioperative mortality or reoperations. Four of the patients were extubated within 24 h, and the other patient was extubated on postoperative day 2. During follow-up, a good semilunar valve and biventricular heart function was present in all 5 patients, and the New York Heart Association functional class was I for all the patients at the time of their latest follow-up (median 31 months, range 8-82 months). The mean echocardiographic gradient of decellularized aortic homografts was 5.4 ± 3.2 mmHg and 11.6 ± 4.2 mmHg for the decellularized pulmonary homografts. Valvular regurgitation was 0 or 0-I for all DHVs. CONCLUSIONS: A single-stage double semilunar valve replacement with DHV has shown promising early results in these 5 very complex cases, providing an additional surgical option after multiple preceding valve procedures in young patients.

A rare mechanism of aortic regurgitation in a young patient.

A 19-year-old male patient was admitted to our institute with dyspnea. His medical history had no rheumatic fever or infective endocarditis. Physical examination revealed a diastolic murmur over the aortic area, rales of bilateral lungs. Bedside transthoracic echocardiography (TTE) revealed a severe aortic regurgitation (AR) without aortic valve stenosis and a moderately dilated left ventricle accompanied by an ejection fraction of 55%. The aortic valve could not be clearly demonstrated as either bicuspid or tricuspid. Congenital AR typically occurs in conjunction with an additional cardiac abnormality or aortic valve stenosis. Furthermore, bicuspid aortic valves are observed in the majority of patients. The aortic valve is created from the truncus ridge of the truncus arteriosus while the embryological development.