Pulmonary valve stenosis in a recipient twin in twin-to-twin transfusion syndrome with successful balloon valvuloplasty after birth: a case report.

BACKGROUND: Pulmonary stenosis (PS) is a congenital heart diseases (CHDs) with a spectrum of stenosis. Monochorionic (MC) twins are at increased risk of CHDs, especially acquired CHDs in twin-twin transfusion syndrome (TTTS). PS/Pulmonary atresia (PA) is a rare coincidence with TTTS. MC twin pregnancies have increased in last decades due to increasing in maternal age and extensive use of assisted reproductive technologies. Therefore, attention to this group is important for heart abnormalities, especially in twins with TTTS. Multiple cardiac abnormalities in MC twins with TTTS are to be expected due to cardiac hemodynamic changes and may be eliminated by Fetoscopic laser photocoagulation treatment. Prenatal diagnosis of PS is necessary given the importance of treatment after birth. CASE PRESENTATION: We here present a case of coexistence of TTTS with PS in a growth restricted recipient twin who successfully treated with balloon pulmonary valvuloplasty in neonatal period. Also, we detected infundibular PS after valvuloplasty that treated with medical therapy (propranolol). CONCLUSIONS: It is important to detect acquired cardiac abnormalities in MC twins with TTTS, and follow them up after birth to determine the need of intervention in neonatal period.

Transcatheter atrial septal defect closure late after completion of biventricular circulation in patients with pulmonary atresia intact ventricular septum or critical pulmonary stenosis.

OBJECTIVE: This study aimed to explore anatomical and hemodynamic features of atrial septal defect, which was treated by transcatheter device closure late after completion of biventricular circulation in patients with pulmonary atresia and intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS). METHODS: We studied echocardiographic and cardiac catheterization data, including defect size, retroaortic rim length, single or multiple defects, the presence of malalignment atrial septum, tricuspid and pulmonary valve diameters, and cardiac chamber sizes, in patients with PAIVS/CPS who underwent transcatheter closure of atrial septal defect (TCASD), and compared to control subjects. RESULTS: A total of 173 patients with atrial septal defect, including 8 patients with PAIVS/CPS, underwent TCASD. Age and weight at TCASD were 17.3 ± 18.3 years and 36.6 ± 13.9 kg, respectively. There was no significant difference in defect size (13.7 ± 4.0 vs. 15.6 ± 5.2 mm, p = 0.317) and the retro-aortic rim length (3.7 ± 4.3 vs. 3.6 ± 0.3.1 mm, p = 0.948) between the groups; however, multiple defects (50% vs. 5%, p < 0.001) and malalignment atrial septum (62% vs. 14%. p < 0.001) were significantly frequent in patients with PAIVS/CPS compared to control subjects. The ratio of pulmonary to systemic blood flow in patients with PAIVS/CPS was significantly lower than that in the control patients (1.2 ± 0.4 vs. 2.0 ± 0.7, p < 0.001); however, four out of eight patients with atrial septal defect associated with PAIVS/CPS had right-to-left shunt through a defect, who were evaluated by the balloon occlusion test before TCASD. The indexed right atrial and ventricular areas, the right ventricular systolic pressure, and mean pulmonary arterial pressure did not differ between the groups. After TCASD, the right ventricular end-diastolic area remained unchanged in patients with PAIVS/CPS, whereas it significantly decreased in control subjects. CONCLUSIONS: Atrial septal defect associated with PAIVS/CPS had more complex anatomy, which would be a risk for device closure. Hemodynamics should be individually evaluated to determine the indication for TCASD because PAIVS/CPS encompassed anatomical heterogeneity of the entire right heart.

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.

Pulmonary artery dissection following pulmonary balloon valvuloplasty in dogs.

INTRODUCTION/OBJECTIVES: Pulmonary artery dissection is a rare complication following balloon valvuloplasty for pulmonic stenosis. We sought to report the rate of this complication in dogs and describe the demographic, clinical, procedural, and outcome data in affected dogs. ANIMALS, MATERIALS AND METHODS: Medical records at a single academic institution between 2002 and 2021 were reviewed for dogs with pulmonic stenosis treated by a balloon valvuloplasty. Dogs were included for evaluation if there was evidence of pulmonary artery dissection on echocardiography or necropsy following balloon valvuloplasty. The demographic, clinical, surgical, and follow-up information were then recorded. RESULTS: Six dogs were included from 210 balloon valvuloplasty procedures for pulmonic stenosis giving a 3.9% rate of pulmonary dissection. There was a variety of signalment, pulmonary valve morphologies, and balloon catheter types used in each dog. All dogs had severe pulmonic stenosis (median pressure gradient of 208 mmHg, range 94-220 mmHg) with 5/6 dogs having a pressure gradient >144 mmHg. The median balloon to pulmonary valve annulus ratio was 1.35 (range 1.25-1.5). Three dogs died perioperatively, and three dogs were alive at follow up 3.3, 4.0, and 4.1 years postoperatively. CONCLUSION: Pulmonary artery dissection is a rare complication following balloon valvuloplasty for pulmonic stenosis. Extreme elevations in preoperative pulmonary valve flow velocity were common. Prognosis is variable, with a potential 50% perioperative survival rate, but extended survival times were noted in those patients discharged from hospital.

Fatal acute lung injury after balloon valvuloplasty in a dog with pulmonary stenosis.

A one-year-old French Bulldog was referred for the management of a severe form of pulmonary valve stenosis (PS) complicated by right-sided congestive heart failure. Echocardiography showed severe valvular PS with right ventricular concentric hypertrophy, dilatation and severe right atrial enlargement. A pulmonary balloon valvuloplasty (PBV) was performed with a balloon-to-pulmonary annulus ratio of 1.36. Echocardiography immediately after PBV showed a significant reduction in right atrial and ventricular size, improved opening and mobility of the pulmonary valve leaflets, and a 75% reduction in the pulmonary pressure gradient from 158 mmHg pre-operative to 40 mmHg post-operative. The dog recovered well from anesthesia, but 2 h later, it suddenly showed severe respiratory distress. Focus cardiac ultrasound showed increased left cardiac size with echocardiographic signs of high left ventricular filling pressure. Bedside lung ultrasound showed diffuse numerous-to-confluent B lines, compatible with a severe alveolar-interstitial syndrome. The dog was treated with furosemide, helmet continuous positive airway pressure, and then mechanical ventilation but without success. At post-mortem evaluation, histological examination of the lung showed diffuse, severe broncho-alveolar edema with mixed leukocyte, fibrin, and red blood cell infiltrate. Moreover, severe congestion and multifocal alveolar hemorrhages were evident. All findings were compatible with fatal acute lung injury after PBV secondary to pulmonary reperfusion-ischemia injury and increased pulmonary capillary hydrostatic pressure. Based on the present case, acute lung injury should be considered as a rare but serious complication of PBV.

Acute pulmonary edema in a dog with severe pulmonary valve stenosis: A rare complication after balloon valvuloplasty.

Pulmonic stenosis is a frequent congenital heart disease in dogs, and the treatment of choice is balloon valvuloplasty which is usually safe and successful. The authors describe for the first time a severe complication after balloon valvuloplasty in a five-month-old dog. After effective treatment, with a considerable drop in right ventricular pressures, the dog developed hypoxemia and dyspnea due to pulmonary edema. The dog underwent intensive care and symptoms improved after a few hours of oxygen therapy, continuous positive airway pressure, and furosemide. Although this event is rare, it could have a large impact on patient survival and should be considered in the treatment of severe pulmonary valve stenosis in the future.

Combined Echo and Fluoroscopy-Guided Pulmonary Valvuloplasty in Neonates and Infants: Efficacy and Safety.

Percutaneous balloon pulmonary valvuloplasty (PBPV) is the treatment of choice for isolated pulmonary valve stenosis. While this procedure is highly efficacious and has an excellent safety profile, as currently practiced, patients are obligatorily exposed to the secondary risks of ionizing radiation and contrast media. To mitigate these risks, we developed a protocol which utilized echo guidance for portions of the procedure which typically require fluoroscopy and/or angiography. Ten cases of echo-guided pulmonary valvuloplasty (EG-PBPV) for isolated pulmonary stenosis in children less than a year of age were compared to a historical cohort of nineteen standard cases using fluoroscopy/angiography alone, which demonstrated equivalent procedural outcomes and safety, while achieving a median reduction in radiation (total dose area product) and contrast load of 80% and 84%, respectively. Our early experience demonstrates that EG-PBPV in neonates and infants has results equivalent to standard valvuloplasty but with less radiation and contrast.

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.

Venous Properties in a Fontan Patient with Successful Remission of Protein-Losing Enteropathy.

We present the case of a 1-year-old boy who developed protein-losing enteropathy (PLE) within 2 months of a fenestrated Fontan procedure. His fenestration rapidly closed despite bilateral pulmonary stenosis (BPS). Subsequent to PLE onset, both fenestration and the bilateral pulmonary artery were reconstructed, and the patient's PLE had been in remission, with additive use of medications, for more than 2 years. Notably, although fenestration closed again and central venous pressure (CVP) reduction was minimal, the surrogates of venous return resistance were markedly suppressed as shown by increased blood volume, reduced estimated mean circulatory filling pressure, and suppressed CVP augmentation against a contrast agent. Taken together, dynamic characteristics of venous stagnation, rather than the absolute value of CVP, were ameliorated by the pulmonary reconstruction and use of medications, suggesting a significant role of venous property in the physiology of PLE. In addition, simultaneous measures of CVP and ventricular end-diastolic pressure during the abdominal compression procedure suggested a limited therapeutic role of fenestration against PLE in this patient.

Semilunar Valve Interventions for Congenital Heart Disease: JACC State-of-the-Art Review.

Transcatheter balloon valvuloplasty for the treatment of aortic and pulmonary valve stenosis was first described nearly 40 years ago. Since that time, the technique has been refined in an effort to optimize acute outcomes while reducing the long-term need for reintervention and valve replacement. Balloon pulmonary valvuloplasty is considered first-line therapy for pulmonary valve stenosis and generally results in successful relief of valvar obstruction. Larger balloon to annulus (BAR) diameter ratios can increase the risk for significant valvar regurgitation. However, the development of regurgitation resulting in right ventricular dilation and dysfunction necessitating pulmonary valve replacement is uncommon in long-term follow-up. Balloon aortic valvuloplasty has generally been the first-line therapy for aortic valve stenosis, although some contemporary studies have documented improved outcomes following surgical valvuloplasty in a subset of patients who achieve tri-leaflet valve morphology following surgical repair. Over time, progressive aortic regurgitation is common and frequently results in the need for aortic valve replacement. Neonates with critical aortic valve stenosis remain a particularly high-risk group. More contemporary data suggest that acutely achieving an aortic valve gradient <35 mm Hg with mild aortic regurgitation may improve long-term valve performance and reduce the need for valve replacement. Continued study will help to further improve outcomes and reduce the need for future reinterventions.

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.

The progression of an acceptable pulmonary stenosis immediately after total correction of tetralogy of Fallot.

BACKGROUND: A certain degree of pulmonary stenosis after total correction of tetralogy of Fallot has been considered acceptable. But the long-term outcomes are not well understood. We observed the natural course of immediate pulmonary stenosis and investigated related factors for progression. METHODS: Fifty-two patients with acceptable pulmonary stenosis immediately after operation were enrolled. Acceptable pulmonary stenosis was defined as peak pressure gradient between 15 and 45 mmHg by Doppler echocardiography. Latent class linear mixed model was used to differentiate patients with progressed pulmonary stenosis, and the factors related to progression were analysed. RESULTS: Pulmonary stenosis progressed in 14 patients (27%). Between the progression group and no progression group, there were no significant differences in operative age, sex, and the use of the transannular patch technique. However, immediate gradient was higher in the progression group (32.1 mmHg versus 25.7 mmHg, p = 0.009), and the cut-off value was 26.8 mmHg (sensitivity = 65.3%, specificity = 65.8%). Main stenosis at the sub-valve was observed more frequently in the progression group (85.7% versus 52.6%, p = 0.027). Despite no difference in the preoperative pulmonary valve z value, the last follow-up pulmonary valve z value was significantly lower in the progression group (-1.15 versus 0.35, p = 0.002). CONCLUSIONS: Pulmonary stenosis immediately after tetralogy of Fallot total correction might progress in patients with immediate pulmonary stenosis higher than ≥26.8 mmHg and the main site was sub-valve area.

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.