30 years' experience with the arterial switch operation: risk of pulmonary stenosis and its impact on post-operative prognosis.

Pulmonary stenosis is one of the most common complications in patients with transposition of the great arteries after the arterial switch operation. The reason for this is multifactorial and related to the anterior shift of the pulmonary trunk during the LeCompte manoeuvre, the complex suture line required to fill the gaps after harvesting the coronary arteries, and the need for patch implantation to maintain a tensionless anastomosis.We reviewed all patients with transposition of the great arteries operated on at our institute between 1991 and 2020 to establish the frequency of pulmonary stenosis during post-operative follow-up, reinterventions, and reoperations related to pulmonary stenosis and its potential risk factors.During the analysed period, we performed 848 arterial switch operations for simple and complex cases of transposition of the great arteries. The overall early mortality was 6.96%, and the late mortality was 2.53%. Among all study groups, 243 (28.66%) patients developed mild pulmonary stenosis, 43 patients (5.07%) developed moderate, and 45 patients (5.31%) developed severe pulmonary stenosis. During follow-up, 21 patients required interventions related to pulmonary stenosis. Pulmonary reconstruction with patches, aortic arch anomalies, and ventricular septal defects associated with transposition of the great arteries were significant risk factors. Nine patients required reoperation because of pulmonary artery stenosis with patch reconstruction of the pulmonary artery, aortic arch anomalies, and aortic cross-clamping time, increasing the risk of reoperation.Pulmonary stenosis in patients with transposition of the great arteries after the arterial switch operation is a common complication. If significant, it occurs early after surgery and is the most frequent reason for post-operative interventions and reoperations.

Neonatal right mini thoracotomy for repair of critical pulmonary stenosis.

Over the last few decades, due to evolving surgical techniques and medical management, there has been a significant decrease in the rate of mortality and complications for neonates born with critical pulmonary valve stenosis. Median sternotomy is the standard approach; however, this longitudinal midline incision is invasive and leaves a significant scar. A right mini thoracotomy approach to this surgical repair decreases recovery time and the chance of possible future psychological distress from a visible median sternotomy scar. This is the first article to describe a right mini thoracotomy approach for critical pulmonary stenosis during the neonatal period.

The Longest Documented Postoperative Survivor of a Brock Procedure 63 Years Later.

An infant with critical pulmonary valve stenosis underwent a Brock procedure in 1957 with subsequent repair of pulmonary stenosis and an atrial septal defect at age 6. At age 17 she underwent repair for paradoxical embolization secondary to a residual atrial septal defect. She presented 54 years later with recurrent pulmonary stenosis and a symptomatic 6.2-cm pulmonary artery aneurysm repaired with a 23-mm aortic homograft. This patient is the longest reported postoperative survivor after a Brock procedure. She continues to do well 63 years after her initial surgery.

Pulmonary ductal coarctation: An entity associated with congenital heart defects involving the right ventricle outflow tract.

We present a case of a prematurely born 2-month-old girl with a double outlet right ventricle with pulmonary atresia and a left pulmonary artery coarctation arising after ductal closure. The case highlights the importance of knowledge and identification of such an anomaly in patients with congenital heart malformations with severe pulmonary stenosis to atresia.

Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot.

Importance: The choice of the right surgical technique for correction of tetralogy of Fallot (TOF) is contentious for patients with a moderate to severe right outflow tract obstruction. The use of a transannular patch (TAP) exposes patients to chronic pulmonary regurgitation, while valve-sparing (VS) procedures may incompletely relieve pulmonary obstruction. Objective: To compare 30-year outcomes of TOF repair after a VS procedure vs TAP. Design, Setting, and Participants: This retrospective population-based cohort study was conducted among all patients with TOF born in the province of Quebec, Canada, from 1980 to 2015 who underwent complete surgical repair. Patients who received a TAP or VS procedure were matched using a propensity score based on preoperative factors in a 1:1 ratio. Data were analyzed from March 2020 through April 2021. Exposures: The study groups were individuals who received TAP and those who received VS. The VS group was further stratified by the presence of residual pulmonary stenosis. Main Outcomes and Measures: The primary outcome was all-cause mortality, with 30-year survival evaluated using Cox proportional-hazards models. Secondary outcomes included the cumulative mean number of cardiovascular interventions, pulmonary valve replacements (PVRs), and cardiovascular hospitalizations were evaluated using marginal means/rates regressions. Results: Among 683 patients with TOF (401 patients who underwent TAP [58.7%] and 282 patients who underwent a VS procedure [41.3%]), adequate propensity score matching was achieved for 528 patients (264 patients who underwent a VS procedure and 264 patients who underwent TAP). Among this study cohort, 307 individuals (58.1%) were men. The median (interquartile range [IQR]) follow-up was 16.0 (8.1-25.4) years, for a total of 8881 patient-years, including 63 individuals (11.9%) followed up for more than 30 years. Individuals who received a VS procedure had an increased 30-year survival of 99.1% compared with 90.4% for individuals who received TAP (hazard ratio [HR], 0.09 [95% CI, 0.02-0.41]; P = .002). Patients who underwent TAP had an increased 30-year cumulative mean number of cardiovascular interventions compared with patients who underwent a VS procedure without residual pulmonary stenosis (2.0 interventions [95% CI, 1.5-2.7 interventions] vs 0.7 interventions [95% CI, 0.5-1.1 interventions]; mean ratio [MR], 0.36 [95% CI, 0.25-0.50]; P < .001) and patients who underwent a VS procedure with at least moderate residual stenosis (1.3 interventions [95% CI, 0.9-1.9 interventions]; MR, 0.65 [0.45-0.93]; P = .02). Results were similar for PVR, with a 30-year cumulative mean 0.3 PVRs [95% CI, 0.1-0.7 PVRs] for patients who underwent a VS procedure without residual pulmonary stenosis (MR, 0.22 [95% CI, 0.12-0.43]; P < .001) and 0.6 PVRs (95% CI, 0.2-1.5 PVRs) for patients with at least moderate residual stenosis (MR, 0.44 [95% CI, 0.21-0.93]; P = .03), compared with 1.4 PVRs (95% CI, 0.8-2.5 PVRs) for the TAP group. No statistically significant difference was found for cardiovascular hospitalizations. Conclusions and Relevance: This study found that patients who underwent a VS procedure had increased 30-year survival, fewer cardiovascular reinterventions, and fewer PVRs compared with individuals who underwent TAP, even in the presence of significant residual pulmonary stenosis. These findings suggest that it is beneficial to perform a VS procedure when possible, even in the presence of moderate residual stenosis, compared with the insertion of a TAP.

High Glucose Metabolism in the Right Ventricular Myocardium Due to Extrinsic Pulmonary Stenosis by Mediastinal Lymphoma.

Acquired pulmonary stenosis in adults is rare and is usually caused by extrinsic compression from a mediastinal tumor. We present a case of anterior mediastinal Hodgkin lymphoma, who presented with cough and hemoptysis. Compression of the bilateral pulmonary arteries by the mediastinal mass was demonstrated by transthoracic echocardiography and CT pulmonary angiography. FDG PET/CT showed diffusely increased FDG uptake in right ventricular myocardium in addition to lymphomatous involvement of the lymph nodes and spleen.

Anatomic Risk Factors for Reintervention After Arterial Switch Operation for Taussig-Bing Anomaly.

BACKGROUND: This study aimed to determine the factors related to reintervention, especially for pulmonary stenosis (PS), in patients with Taussig-Bing anomaly (TBA) after the arterial switch operation. METHODS: This retrospective study included 34 patients with TBA who underwent the arterial switch operation between 1993 and 2018. Preoperative anatomic and physiologic differences and long-term outcomes were determined using a case-matched control with transposition of the great arteries with ventricular septal defect and TBA with an anterior and rightward aorta. RESULTS: At arterial switch operation, median age was 43 days (range, 16 to 102) and median body weight was 3.6 kg (range, 2.8 to 3.8 kg). Aortic arch obstruction and coronary anomalies were present in 64% and 41% of patients, respectively. The hospital mortality rate was 11%, including one cardiac death, and the late mortality rate was 2.9%. Furthermore, 41% patients underwent 26 reinterventions for PS. Patients undergoing PS-related reintervention had a significantly larger native pulmonary artery to aortic annulus size ratio than patients not receiving reintervention (1.69 vs 1.41, P = .02). This ratio was the only predictor of PS-related reintervention; it was significantly higher in the TBA group than in the transposition of great arteries/ventricular septal defect group. The PS-related reintervention was required more in the TBA group than in the transposition of great arteries/ventricular septal defect group. CONCLUSIONS: Regardless of complex coronary anatomy and associated anomalies, early and late survival were acceptable. Postoperative PS was strongly associated with having a larger native pulmonary valve, suggesting that an optimal surgical reconstruction was required for achieving an appropriate aortopulmonary anatomic relationship during the arterial switch operation.

Surgical Repair of Peripheral Pulmonary Artery Stenosis in Patients Without Williams or Alagille Syndromes.

Peripheral pulmonary artery stenosis is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients. This was a retrospective review of 30 patients who underwent surgical repair for peripheral pulmonary artery stenosis. Concomitant anatomical diagnoses in 20 patients (67%) included supravalvar aortic stenosis (n = 8), tetralogy of Fallot (n = 4), d-transposition of the great arteries (n = 2), truncus arteriosus (n = 2), hypoplastic left heart syndrome (n = 2), ventricular septal defect (n = 1), and patent ductus arteriosus (n = 1). Additional medical diagnoses in 15 patients (50%) included elastin arteriopathy (n = 9), pulmonary artery calcinosis (n = 1), arterial tortuosity syndrome (n = 1), DiGeorge syndrome (n = 1), and Noonan syndrome (n = 1). Median age at surgery was 3.6 years (interquartile range 1.6-7.4 years). Seventeen patients (57%) had prior cardiac operations, and 16 patients (53%) previously underwent percutaneous intervention. With surgery, mean right ventricle-to-aortic systolic pressure ratio decreased from 0.95 ± 0.2 to 0.28 ± 0.08 (P< 0.0001). Median duration of cardiopulmonary bypass was 369 minutes. There was 100% survival to hospital discharge, with no mortality at mean follow-up of 2.3 years. No patient required reoperation, while 4 underwent balloon dilation. Freedom from pulmonary artery catheter-based reintervention was 95% and 80% at 12 and 36 months, respectively. Patients with peripheral pulmonary artery stenosis without either Williams or Alagille syndrome can successfully undergo surgical repair with a significant reduction in right ventricle-to-aortic pressure ratios.

A stented bovine pericardial prosthesis in the pulmonary position.

BACKGROUND: Pulmonary valve replacement is very common among patients with congenital heart disease. The Carpentier Edwards Perimount valve (Edwards Lifesciences, Irvine, Calif), which was originally designed for the aortic position is among the most implanted valves. We aim to describe the follow-up of this valve in the pulmonary position. METHODS: Patients with a Perimount valve implanted between 2003 and 2013 in the University Medical Center Groningen were followed for the primary end point reintervention, defined as surgical or transcatheter valve replacement. Secondary end point was the occurrence of valve failure, defined as significant valvular regurgitation or stenosis. Explanted valves were histologically examined. RESULTS: Forty-five patients (median age at operation 27.8 years, 55.6% women) had a mean follow-up duration of 5.8 ± 3.3 years. There were 7 reinterventions (5 surgical and 2 transcatheter). Freedom from reintervention was respectively 95% ± 4% and 83% ± 8% at 5- and 10- years of follow-up. Freedom from valve failure was 75% ± 4% at 2 years, 65% ± 8% at 5 years of follow-up and 57% ± 10% at 10 years of follow-up. Morphology evaluation (n = 4) showed stiffened valves in the open position, with extensive fibrous tissue overgrowth on the leaflets and a variable proliferation of myofibroblasts. CONCLUSIONS: The Perimount valve has adequate function in the pulmonary valve position at 5 years of follow-up, although after 10 years of follow-up valve failure and reinterventions are common. Explanted valves show retraction and stiffening of the leaflets due to a fibrotic layer on both sides of the leaflet.

Fetal and Neonatal Circulatory Disorders in Twin to Twin Transfusion Syndrome (The Secondary Publication).

Twin to twin transfusion syndrome (TTTS) is a major complication of monochorionic diamniotic (MD) twins, and its onset is known to be associated with placental vascular anastomoses and blood flow imbalance. In a typical case of TTTS, the recipient develops polyhydramnios, weight gain, cardiomegaly and hydrops fetalis in the uterus. In contrast, the donor develops oligohydramnios and intrauterine growth restriction. Recently, the significance of the renin-angiotensin-aldosterone system (RAAS) that transfers from the donor to the recipient has attracted interest in the fetal circulation of TTTS. The donor has decreased renal blood flow due to decreased circulating blood volume. For this reason, the secretion of RAAS hormones is augmented in the fetal kidneys of the donor. In TTTS, these RAAS hormones from the donor transfer to the recipient through the anastomosed vessels. In addition to excess preload, the recipient heart is exposed to excess afterload due to systemic vasoconstriction through RAAS hormones. Commonly occurring complications in the recipient include myocardial hypertrophy, atrioventricular valve regurgitation, and pulmonary valve stenosis or pulmonary atresia. Fetoscopic laser photocoagulation (FLP) has been introduced recently because neither mortality nor neurological morbidity have been satisfactorily improved with conventional treatment. FLP is a curative method that may improve the prognosis of TTTS. In Japan, this procedure has been performed frequently, and positive neurological outcomes have been achieved.

Partial Sternectomy to Prevent Rastelli Conduit Compression in Patients With Conotruncal Anomalies.

This report describes 2 cases of redo cardiac surgery in adult patients with conotruncal anomalies (double-outlet right ventricle and transposition of the great arteries) with pulmonary stenosis who underwent the Rastelli procedure in childhood. Although the Rastelli operation is one of the best options for the patients, conduit stenosis has occasionally developed secondary to compression by the anterior chest wall. To prevent compression by the sternum, partial sternectomy with Rastelli conduit change was performed in these 2 patients.

Outcomes of Reinterventions for Children with Postoperative Pulmonary Venous Restenosis.

This report aims to summarize and evaluate the efficacy and experience of reintervention for children with postoperative pulmonary venous restenosis. Clinical data from 61 patients with postoperative pulmonary venous restenosis who underwent reintervention at Shanghai Children's Medical Center (SCMC) from September 2009 to June 2018 were retrospectively analyzed. The patients comprised 38 boys and 23 girls, with a mean age of 15.4 ± 12.6 months (2-83 months) and a mean weight of 8.1 ± 3.4 kg (3.7-18.5 kg). The mean pulmonary venous velocity was 2.31 ± 0.47 m/s (1.86-3.22 m/s). Primary disease included 48 cases of total anomalous pulmonary venous drainage, nine cases of partial anomalous pulmonary venous drainage, and four cases of primary pulmonary venous stenosis. The reintervention procedures included 34 cases using the sutureless technique, ten cases using bovine pericardium enlargement, three cases using blunt enlargement, four cases of balloon dilatation, one case using stent implantation and nine cases involving more than two surgical methods. The early postoperative pulmonary venous velocity was 1.16 ± 0.20 m/s. There were five in-hospital deaths, resulting in a mortality rate of 8.2%. Fifty-six survivors were followed for 52.8 ± 46.5 months (6-103 months) with no delayed deaths. Echocardiography showed pulmonary venous anastomosis and diameter growth after reintervention, exhibiting a mean growth speed of 0.026 ± 0.013 cm/month (p < 0.05) and a mean velocity of 1.24 ± 0.26 m/s; five patients experienced varying degrees of pulmonary venous obstruction (> 1.6 m/s), but did not require reoperation. Postoperative pulmonary venous restenosis is a common complication after surgery for pulmonary venous malformations. Reintervention should be performed in the early period of pulmonary venous obstruction. Growth of pulmonary venous anastomoses was observed after performing the sutureless technique, bovine pericardium enlargement and blunt enlargement. Although balloon dilatation has a good effect in the early postoperative period, its restenosis rate is high, and strict mid- to long-term follow-up is needed.

Improving outcome of valve replacement for carcinoid heart disease.

OBJECTIVE: Carcinoid heart disease is characterized by tricuspid valve regurgitation and varying degrees of pulmonary valve regurgitation or stenosis. Valve replacement procedures may be complicated by systemic effects of carcinoid syndrome, as well as hepatic dysfunction and right heart failure. This study was performed to identify factors that might be associated with improving early mortality rates and late outcomes. METHODS: Between November 1985 and January 2018, 240 adult patients underwent surgery for carcinoid heart disease at the Mayo Clinic. We analyzed the association of multiple clinical and echocardiographic variables on early mortality and late survival. RESULTS: The median (interquartile range) age of patients was 63 years (55-69), and 117 patients (49%) were male. Before operation, 157 patients (70%) had New York Heart Association class III or IV limitation. Somatostatin analogs were used in 221 patients (92%), and long-acting somatostatins were used in 130 patients (54%). Loop diuretic therapy was used preoperatively in 125 patients (52%). Early mortality rate was 29% (9/22) between 1985 and 1994, but decreased to 7% (6/81) during 1995 to 2004, and to 5% (7/128) from 2005 onward. Overall survival estimates at 1, 3, and 5 years were 69%, 48%, and 34%, respectively. Older age, advanced New York Heart Association class, and a nonlinear effect of creatinine were independently associated with overall mortality. CONCLUSIONS: Valve replacement for carcinoid heart disease has acceptable short-term mortality, and early risk has decreased in the current era. Earlier intervention may improve overall survival.

Transcatheter pulmonary valve replacement using the melody valve for treatment of dysfunctional surgical bioprostheses: A multicenter study.

BACKGROUND: Stented bioprosthetic valves (BPVs) are commonly used for surgical pulmonary valve (PV) replacement in postoperative congenital heart disease, but develop structural failure in a time-related fashion. The Melody transcatheter PV (TPV) (Medtronic, Minneapolis, Minn) has been used to treat BPV dysfunction, but there have been few studies in this population. METHODS: We performed a retrospective, multicenter study to evaluate Melody valve function in patients who underwent TPV replacement (TPVR) into a dysfunctional pulmonary BPV. RESULTS: One hundred patients who underwent TPVR at 10 centers between January 2010 and June 2015 were enrolled. The median patient age was 22 years (range, 5-79 years), and 32 patients were age <18 years. The underlying diagnosis was tetralogy of Fallot in 80 patients, and moderate or severe pulmonary regurgitation (PR) was present in 84%. The TPV was implanted into various types of BPVs, with a median size of 23 mm (range, 19-33 mm). At hospital discharge, PR was mild or less in all but 1 patient, and the mean Doppler right ventricular outflow tract (RVOT) gradient was reduced from a mean of 29.3 ± 12.0 mm Hg to 16.2 ± 6.9 mm Hg (median, 29 mm Hg to 16 mm Hg; P < .001). During follow-up (median, 12.4 months), no patients underwent reintervention on the TPV. Endocarditis was diagnosed in 1 patient who was managed medically without intervention. The mean RVOT gradient at the most recent follow-up was ≤35 mm Hg in all patients, and was similar to that at early postimplantation. PR was more than mild in only 1 patient. Hemodynamic outcomes did not differ between patients with small BPVs (≤23 mm) and those with large BPVs (≥25 mm). CONCLUSIONS: TPVR restores competence and relieves the obstruction of dysfunctional surgical BPVs, with excellent early results in both small and large BPVs, highlighting the potential for TPVR to extend the life of existing BPVs in adults and children. Collaboration between surgeons and cardiologists is important to determine the optimal lifetime management, combining surgical PV replacement and TPVR in this population.

Results of balloon pulmonary valvoplasty in children with Noonan's syndrome.

Pulmonary valve stenosis is common in patients with Noonan's syndrome. The response to balloon valvoplasty varies.We assessed the correlation between re-intervention rate, immediate response, and the progress of the valve gradient over time after intervention. METHODS: This is a retrospective study conducted from 1995 to 2014. RESULTS: Of 14 patients identified, seven had re-intervention 28±54 months (range 3-149, median 3.3) after valvoplasty. These patients did not have a significant decrease in gradient after intervention. Their gradient subsequently decreased during follow-up and then became static before increasing years after intervention. In contrast, the gradient of patients not requiring further intervention continually reduced over time. Demographics did not differ between these groups. CONCLUSION: We could not identify predisposing factors for long-term success of pulmonary valvoplasty in Noonan's patients, but the trajectory of gradients differs significantly between patients needing re-intervention from those who remain free from re-intervention.

Pulmonic Valve Disease: Review of Pathology and Current Treatment Options.

PURPOSE OF REVIEW: Our review is intended to provide readers with an overview of disease processes involving the pulmonic valve, highlighting recent outcome studies and guideline-based recommendations; with focus on the two most common interventions for treating pulmonic valve disease, balloon pulmonary valvuloplasty and pulmonic valve replacement. RECENT FINDINGS: The main long-term sequelae of balloon pulmonary valvuloplasty, the gold standard treatment for pulmonic stenosis, remain pulmonic regurgitation and valvular restenosis. The balloon:annulus ratio is a major contributor to both, with high ratios resulting in greater degrees of regurgitation, and small ratios increasing risk for restenosis. Recent studies suggest that a ratio of approximately 1.2 may provide the most optimal results. Pulmonic valve replacement is currently the procedure of choice for patients with severe pulmonic regurgitation and hemodynamic sequelae or symptoms, yet it remains uncertain how it impacts long-term survival. Transcatheter pulmonic valve replacement is a rapidly evolving field and recent outcome studies suggest short and mid-term results at least equivalent to surgery. The Melody valve® was FDA approved for failing pulmonary surgical conduits in 2010 and for failing bioprosthetic surgical pulmonic valves in 2017 and has been extensively studied, whereas the Sapien XT valve®, offering larger diameters, was approved for failing pulmonary conduits in 2016 and has been less extensively studied. Patients with pulmonic valve disease deserve lifelong surveillance for complications. Transcatheter pulmonic valve replacement is a novel and attractive therapeutic option, but is currently only FDA approved for patients with failing pulmonary conduits or dysfunctional surgical bioprosthetic valves. New advances will undoubtedly increase the utilization of this rapidly expanding technology.

The effect of balloon valvuloplasty for bioprosthetic valve stenosis at pulmonary positions.

BACKGROUND: Balloon dilatation of a bioprosthetic valve in the pulmonary position could be performed to delay valve replacement. We proposed to identify the long-term effectiveness of such a procedure. METHODS: We reviewed the medical records of 49 patients who underwent balloon valvuloplasty between January 2000 and December 2015. The primary goal was to determine the time interval until the following surgical or catheter intervention. RESULTS: The mean age at bioprosthetic valve insertion was 5.7 years old, and the mean age for ballooning was 11.7 years. The mean interval after pulmonary valve replacement was 71.6 months. The mean ratio of balloon size to valve size was 0.94. The pressure gradient through the pulmonary valve after balloon valvuloplasty was significantly improved (55.3 ± 18.5 mm Hg vs 33.8 ± 21.5 mm Hg, P < .001). There were no significant changes in pulmonary regurgitation and no serious adverse events. Patients had a mean freedom from re-intervention of 30.6 months after balloon valvuloplasty. The interval of freedom from re-intervention was affected only by the pressure gradient before balloon valvuloplasty and the patient age at insertion. The mean interval to re-intervention in patients with pressure gradients less than 48.5 mm Hg before ballooning was 46.0 months, which was significantly longer than for those with a higher gradient (18.7 months). CONCLUSION: The effectiveness of this process may depend on the pressure gradient before ballooning and the patient age at valve insertion. It is possible that earlier valvuloplasty at pressure gradient not over 48.5mm Hg may have a benefit to delaying re-operation.

Again, Two Melodies in Concert: Transcatheter Double Valve Replacement in Hedinger Syndrome.

Transcatheter pulmonary valve implantation is established as a valuable option to reconstruct failing right ventricular outflow tract function. Percutaneous tricuspid valve-in-valve or valve-in-ring reconstruction is even applied with increasing acceptance. A 46-year-old woman with a diagnosis of carcinoid-dependent right heart failure underwent surgical bioprosthetic tricuspid and pulmonary valve replacement. Almost 1 year later, she presented again with markedly dilatated and reduced right heart function caused by degeneration of both biologic valves. We report a successful two-stage percutaneous transcatheter double-valve replacement with the use of a Melody valve in pulmonary and tricuspid positions.