Bicuspidisation of unicuspid stenotic pulmonary valve in a nine-year-old male.

We report the case of a 9-year-old male with severe congenital pulmonary valve stenosis referred to our centre for percutaneous valvotomy. On admission, trans-thoracic echocardiogram confirmed a unicuspid pulmonary valve with a peak/mean pulmonary valve gradient of 91/53 mmHg and a pulmonary annulus of 13.8 mm (-0.8 Z Score). It also showed an enlarged RV (RV/LV ratio 0,9). During cardiac catheterisation, an additional atrial septal defect (secundum) with significant left to right shunt (Qp/Qs > 2) was diagnosed, which was not amenable to percutaneous closure. The patient was referred for surgical repair.The atrial septal defect was closed by a direct running suture. The repair of the unicuspid valve consisted in bicuspidisation by a large commissurotomy to the left anterior wall of the pulmonary artery. The neo-commissure was created with two separate patches of autologous pericardium secured to the wall of the pulmonary root. The adjustment of the effective height of the pulmonary valve leaflets was done by trimming the patches and a triangular plication of the newly created posterior leaflet. Perioperative echocardiogram showed a peak gradient of 15 mm Hg and trivial pulmonary regurgitation. The total cross-clamp time was 92 min and the bypass time 123 min with a favourable evolution after the surgery.The particularity of the case is represented by the complexity of the bicuspidisation procedure. Using this technique, a tailored approach is needed for every patient.

Percutaneous pulmonary valve implantation in a patient with congenitally corrected transposition of the great arteries: a case report.

BACKGROUND: Percutaneous pulmonary valve implantation has become an attractive method of dysfunctional right ventricle outflow tract treatment. CASE PRESENTATION: We describe a unique case of a 20-year-old Caucasian male patient with a complex cyanotic heart defect, namely pulmonary atresia, with congenitally corrected transposition of the great arteries and ventricular septal defect after Rastelli-like surgery at the age of 5 years with homograft use. At the age of 20 years, the patient needed percutaneous pulmonary valve implantation owing to homograft dysfunction. Despite unusual course of the coronary arteries, balloon testing in the landing zone of the right ventricle outflow tract excluded potential coronary artery compression. Then, after presentation, a Melody valve was implanted successfully in the pulmonary valve position. The 8-year follow-up was uneventful. CONCLUSION: This is likely the first description of a percutaneous pulmonary valve implantation in such anatomy. Such a procedure is feasible; however, it requires exceptional caution owing to the anomalous coronary arteries course, which can be the reason for their compression.

Pulmonary Valve Stenosis: From Diagnosis to Current Management Techniques and Future Prospects.

Pulmonary stenosis (PS) is mainly a congenital defect that accounts for 7-12% of congenital heart diseases (CHD). It can be isolated or, more frequently, associated with other congenital defects (25-30%) involving anomalies of the pulmonary vascular tree. For the diagnosis of PS an integrated approach with echocardiography, cardiac computed tomography and cardiac magnetic resonance (CMR) is of paramount importance for the planning of the interventional treatment. In recent years, transcatheter approaches for the treatment of PS have increased however, meaning surgery is a possible option for complicated cases with anatomy not suitable for percutaneous treatment. The present review aims to summarize current knowledge regarding diagnosis and treatment of PS.

Natural history of aortic root dilatation and pathologic aortic regurgitation in tetralogy of Fallot and its morphological variants.

OBJECTIVE: We sought to characterize the natural history of aortic root dilatation and aortic regurgitation in tetralogy of Fallot (TOF). METHODS: A single-center review of patients who underwent TOF repair from January 1960 to December 2022 was performed. Morphology was categorized as TOF-pulmonary stenosis or TOF-variant (including TOF-pulmonary atresia and TOF-pulmonary atresia-major aortopulmonary collateral arteries). Echocardiographically determined diameters and derived z scores were measured at the annulus, sinus of Valsalva, and sinotubular junction immediately before TOF repair and throughout follow-up. Linear mixed-effects models assessed trends in dimensions over time. RESULTS: Of 2205 patients who underwent primary repair of TOF at a median age of 4.9 months (interquartile range, 2.3-20.5 months) and survived to discharge, 1608 (72.9%) patients had TOF-pulmonary stenosis and 597 (27.1%) patients had TOF-variant. At a median postoperative follow-up of 14.4 years (interquartile range, 3.3-27.6 years; range, 0.1-62.6 years), 313 (14.2%) patients had mild or greater aortic regurgitation and 34 (1.5%) patients required an aortic valve or root intervention. The overall mean rates of annular, sinus of Valsalva, and sinotubular junction growth were 0.5 ± 0.2, 0.6 ± 0.3, and 0.7 ± 0.5 mm/year, respectively. Root z scores remained stable with time. At baseline, patients with TOF-variant had larger diameters and z scores at the annulus, sinus of Valsalva, and sinotubular junction, compared with patients with TOF-pulmonary stenosis (all P values < .05). Over time, patients with TOF-variant demonstrated relatively greater annular (P = .020), sinus of Valsalva (P < .001), and sinotubular junction (P < .001) dilatation. Patients with ≥75th percentile root growth rates had a higher incidence of mild or greater aortic regurgitation (P < .001), moderate or greater aortic regurgitation (P < .001), and aortic valve repair or replacement (P = .045). CONCLUSIONS: Patients with TOF-variant are at comparatively greater risk of pathologic root dilatation over time, warranting closer longitudinal follow-up.

Prevalence and determinants of tricuspid regurgitation after repair of tetralogy of Fallot.

BACKGROUND: The prevalence and determinants of tricuspid regurgitation (TR) in patients with repaired Tetralogy of Fallot (rTOF) remain incompletely understood. OBJECTIVES: To explore the prevalence of and factors associated with TR in patients with rTOF, specifically, the relationship of right ventricular (RV) dilatation with TR severity. METHODS: Patients (≥17 yrs) with rTOF referred to our service (2000-2019) were identified. Those with severe pulmonary stenosis, significant shunt, or previous tricuspid valve surgery were excluded. Using standard cardiac MRI protocols, RV, right atrial (RA) and tricuspid valve (TV) parameters were measured and compared. RESULTS: 68 consecutively eligible patients with rTOF were included in the study (27 ± 9 yrs., 35% female). Despite substantial RV volume overload (mean RVEDVi 153 mL/m2), the majority of the cohort (78%) had no or only mild TR. RA volumes, tenting height/area and annular diameter were normal (4.9 ± 2.0 mm, 1.1 ± 1.0 cm2 and 32.4 ± 6.2 mm, respectively). There was no significant correlation of TR fraction with RVEDVi (r = 0.13; p = 0.30), RVEF (r = 0.09; p = 0.44) or tricuspid annular diameter (r = 0.07; p = 0.62). Only RAVi showed a weak but significant correlation with TR fraction (0.29; p = 0.03). In a pooled cohort analysis, including both rTOF patients and adults with a dilated RV from pre-tricuspid shunt lesions, only rTOF was independently associated with higher TR fraction (p = 0.017). CONCLUSION: Despite substantial RV dilatation in a cohort with rTOF, there was surprisingly little TR. We found poor correlation between RVEDVi, RA volumes, tricuspid annular dilatation and the presence of significant TR. These findings question commonly held notions regarding the pathophysiology of functional TR in these patients.

Right Heart Failure in a Patient with Critical Pulmonary Stenosis, Absent Right Pulmonary Artery, and Lung Cancer.

BACKGROUND Unilateral absence of a pulmonary artery (UAPA) is a rare congenital cardiovascular malformation. More than half of UAPA cases have other cardiac lesions such as tetralogy of Fallot or septal defects. Clinical manifestations are diverse and range from heart failure after birth to an incidental finding on chest imaging during adulthood. Whereas early surgical revascularization is recommended in infancy, this is usually not feasible in the adult population. Management in these patients is aimed at treating the complications of UAPA. CASE REPORT A 67-year-old woman was evaluated for subacute right heart failure. An echocardiogram revealed pulmonary stenosis, tricuspid regurgitation, and depressed right ventricular function. Chest computed tomography (CT) showed absence of the right pulmonary artery. Additionally, there was a lung tumor in the right upper lobe. Right-heart catheterization confirmed a critically obstructed pulmonary orifice shown by hemodynamic collapse when crossing the pulmonary valve with the catheter. The patient underwent pulmonary valve balloon dilatation with right ventricular outflow tract stenting followed by percutaneous implantation of a balloon-expandable stent-valve. The clinical course was complicated by a complete heart block. Oncologic management consisted of stereotactic radiotherapy. CONCLUSIONS The combination of UAPA, pulmonary stenosis, and lung cancer is rare. Pulmonary stenosis worsens prognosis in adult patients with UAPA, but also constitutes a therapeutic target. The decision to treat the pulmonary stenosis should be based on the severity of stenosis, the degree of pulmonary hypertension, and individual anatomy. We chose percutaneous pulmonary valve implantation because our patient had a critical pulmonary stenosis with normal pulmonary pressures.

Levoatriocardinal vein in a patient with transposition of great arteries and pulmonary stenosis.

We report a case of a 3-month-old boy transposition of great arteries, ventricular septal defect, and pulmonary stenosis where computed tomography (CT) angiography detected a "levoatriocardinal vein" connecting the left superior pulmonary vein and the left brachiocephalic vein with a preserved normal connection between the left superior pulmonary vein and left atrium. This case highlights the hemodynamic implications of a "levoatriocardinal vein" as well as the role of CT angiography in detecting such associated cardiovascular anomalies in patients with complex congenital heart defects.

Norwood procedure for hypoplastic left heart syndrome associated with valvular pulmonary stenosis.

Association between hypoplastic left heart syndrome and valvular pulmonary stenosis is very rare. Severity of valvular pulmonary stenosis in this setting limits management options. Consequently, patients with this condition are considered poor candidates for Norwood stage one reconstruction. Herein, we describe a newborn with hypoplastic left heart syndrome and significantly dysplastic pulmonary valve who successfully underwent the Norwood procedure with neoaortic valve reconstruction. Therefore, the Norwood procedure with neoaortic valve reconstruction might be an option for this difficult condition.

Discordant Atrioventricular Connections in Situs Inversus, Dextrocardia with Double Outlet Right Ventricle, Pulmonary Stenosis, and Anomalous Coronary Artery: A Rare Case Report.

We report a case of a 2-year-old female presenting with cyanosis since birth and having rare cardiac anatomy of visceral situs inversus, dextrocardia, discordant atrioventricular connections with double outlet right ventricle, atrial communication, subaortic interventricular communication, anteriorly leftward aorta with right aortic arch, single coronary artery with a branch crossing right ventricle outflow tract, pulmonary stenosis, and bilateral superior vena cava. Corrective repair with atrial switch (Senning technique), intracardiac baffle, and right ventricle outflow reconstruction was successfully performed with challenges from altered anatomical location and orientation of the heart and abnormal coronary artery.

Blood cyst of the pulmonary valve causing right ventricular outflow tract obstruction: A case report.

Blood cysts are rare cardiac tumors, often involve the atrioventricular valves. Blood cysts are usually small and tend to be clinically silent. Here, we report a case of blood cyst adhered to the junction of the pulmonary valve and the right ventricular outflow, causing severe outflow obstruction. This case was initially misdiagnosed as pulmonary valve stenosis by transthoracic echocardiography (TTE) and even underwent percutaneous balloon pulmonary valvuloplasty (PBPV). As the right ventricle-to-pulmonary artery peak gradient did not decrease after PBPV procedure, the boy underwent TTE again and the diagnosis was corrected as blood cyst. The cystic mass was successfully resected subsequently, and the boy recovered well.

[Giant Pulmonary Artery Aneurysm Associated with Pulmonary Valve Regurgitation and Stenosis Due to Quadricuspid Pulmonary Valve:Report of a Case].

A 64-year-old woman was diagnosed with dilatation of the pulmonary artery and pulmonary valve stenosis approximately 10 years ago. At the age of 63, she developed hemoptysis and was referred to our hospital. The pulmonary trunk was enlarged to 63 mm with moderate pulmonary valve insufficiency. The transpulmonary valve pressure gradient was 25 mmHg;thus, surgery was performed. A median sternotomy revealed a markedly dilated pulmonary trunk growing into the pericardial cavity. After opening the patient's pulmonary trunk to check the pulmonary valve, a thickened and shortened quadricuspid valve was observed. We replaced the pulmonary valve with a bioprosthetic valve and used a vascular prosthesis to reconstruct the pulmonary artery. The postoperative course was uneventful, and she was discharged 22 days after the surgery. Histopathological examination of the pulmonary artery aneurysm wall revealed cystic medial necrosis.

Sub-pulmonic stenosis caused by a right ventricular outflow tract vegetation in a children with restricted ventricular septal defect.

Infective endocarditis involving the right heart is rarely observed in the pediatric population. Echocardiography plays an important role in its diagnosis, and surgery is indicated in patients with heart failure and persistent sepsis not responding to medical treatment. Here, we report a rare case of restricted ventricular septal defect complicated by a vegetation developed in the right ventricular outflow tract and causing sub-pulmonic stenosis in a 3-year-old male child.

Pulmonary root translocation in corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis with dextrocardia.

We present 13 years old with history of cyanosis and diagnosis of congenitally corrected transposition of great arteries with ventricle septal defect and pulmonary stenosis with dextrocardia. He underwent modified Senning procedure with pulmonary root translocation. Postoperative recovery was uneventful and was extubated on postoperative day 1. Patient remained in sinus rhythm. Postoperative echocardiography showed good biventricular function with no baffle obstruction and mild pulmonary regurgitation with no significant left and right outflow tract obstruction. Patient was discharged on postoperative day 6 and remained asymptomatic on follow up at 3 months.

Case of calcific tricuspid and pulmonary valve stenosis.

Isolated right-sided valvular disease is a much less recognised entity when compared with left-sided valvular heart disease. Almost all the cases of combined pulmonary valve with tricuspid valve involvement are a consequence of underlying carcinoid heart disease. Moreover, severe calcification of tricuspid valve is an extremely unusual finding. We report a case of a severe calcific tricuspid valve stenosis along with severe pulmonary valve stenosis where the exact aetiology could not be established. On reviewing the literature, we did not find any reports describing such a morphology.

Tricuspid Intervention Following Pulmonary Valve Replacement in Adults With Congenital Heart Disease.

BACKGROUND: Tricuspid regurgitation (TR) is common among adults with corrected tetralogy of Fallot (TOF) or pulmonary stenosis (PS) referred for pulmonary valve replacement (PVR). Yet, combined valve surgery remains controversial. OBJECTIVES: This study sought to evaluate the impact of concomitant tricuspid valve intervention (TVI) on post-operative TR, length of hospital stay, and on a composite endpoint consisting of 7 early adverse events (death, reintervention, cardiac electronic device implantation, infection, thromboembolic event, hemodialysis, and readmission). METHODS: The national Canadian cohort enrolled 542 patients with TOF or PS and mild to severe TR who underwent isolated PVR (66.8%) or PVR+TVI (33.2%). Outcomes were abstracted from charts and compared between groups using multivariable logistic and negative binomial regression. RESULTS: Median age at reintervention was 35.3 years. Regardless of surgery type, TR decreased by at least 1 echocardiographic grade in 35.4%, 66.9%, and 92.8% of patients with pre-operative mild, moderate, and severe insufficiency. In multivariable analyses, PVR+TVI was associated with an additional 2.3-fold reduction in TR grade (odds ratio [OR]: 0.44; 95% confidence interval [CI]: 0.25 to 0.77) without an increase in early adverse events (OR: 0.85; 95% CI: 0.46 to 1.57) or hospitalization time (incidence rate ratio: 1.17; 95% CI: 0.93 to 1.46). Pre-operative TR severity and presence of transvalvular leads independently predicted post-operative TR. In contrast, early adverse events were strongly associated with atrial tachyarrhythmia, extracardiac arteriopathy, and a high body mass index. CONCLUSIONS: In patients with TOF or PS and significant TR, concomitant TVI is safe and results in better early tricuspid valve competence than isolated PVR.

Left main coronary artery compression by the right pulmonary artery in a patient with congenital pulmonic stenosis / Compresión del tronco coronario izquierdo por la arteria pulmonar derecha en un paciente con estenosis pulmonar congénita

Abstract Congenital pulmonary stenosis (PS) can be associated with pulmonary artery (PA) dilatation. In some cases, this can cause compression of nearby structures including the left main coronary artery (LMCA). This compression causes angina and is considered an indication for surgical treatment. We present the case of a patient with PS and angina secondary to LMCA compression by the right PA and review the main indications and options for surgical treatment.

Resumen La estenosis pulmonar congénita se asocia a dilatación de la arteria pulmonar. En algunos casos esto puede causar compresión de las estructuras adyacentes incluyendo el tronco de la coronaria izquierda. Esta compresión causa angina y es considerada una indicación para tratamiento quirúrgico. Presentamos el caso de un paciente con estenosis pulmonar y angina secundaria a compresión del tronco de la coronaria izquierda por la arteria pulmonar derecha y revisamos las indicaciones y opciones de tratamiento quirúrgico.

Aortic and Pulmonary Valve Reconstruction Using Autologous Pericardium in Narrow Annuli.

A 71-year-old woman with pulmonary artery aneurysm had a complication of pulmonary valve stenosis with estimated right ventricular pressure 135 mm Hg and severe aortic valve stenosis with peak pressure gradient 112 mmHg. Coronary angiography revealed stenosis of the left coronary arterial system. Because she presented with narrow pulmonary annulus (16.7 mm) and aortic annulus (19.7 mm), we decided to perform aortic and pulmonary valve reconstruction using autologous pericardium. We performed aneurysmorrhaphy of the pulmonary artery, dual-valve reconstruction, and coronary artery bypass grafting. Postoperative echocardiography showed good opening of dual valves without regurgitation; estimated right ventricular pressure was 23 mm Hg.

Intravenous Sotalol for the Treatment of Ventricular Dysrhythmias in an Infant on Extracorporeal Membrane Oxygenation.

Sotalol is a class III anti-arrhythmic agent with beta receptor blocking properties. Intravenous (IV) sotalol may be useful to treat refractory atrial and ventricular arrhythmias. A report on the efficacy and safety of IV sotalol in an infant on extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy (CRRT), who developed refractory ventricular arrhythmias following surgery for congenital heart disease. A 10-day old infant with severe pulmonary valve stenosis underwent surgical pulmonary valvectomy and enlargement of the main pulmonary artery. Post-operatively, the patient developed hemodynamically significant accelerated idioventricular rhythm which was not responsive to a combination of amiodarone, lidocaine, and procainamide leading to 2 cardiac arrest events and placement on ECMO. The amiodarone infusion was uptitrated to 20 mcg/kg/min, but episodes of the hemodynamically compromising arrhythmia continued. Amiodarone was discontinued and IV sotalol was initiated at 42 mg/m2/day, divided to 3 doses, and administered every 8 h, which completely suppressed the arrhythmia. The initial sotalol dose was calculated based on a daily dose of 90 mg/m2 and reduced by an age-related factor as recommended by the FDA approved prescribing information. Subsequently, acute kidney injury requiring CRRT developed. The patient remained on IV sotalol for 3 weeks and then transitioned to oral sotalol. The oral dose was increased to 44 mg/m2/day (3.5 mg every 8 h) to account for the difference in bioavailability between the IV and oral formulations. Serial sotalol levels during IV and PO therapy remained therapeutic on ECMO and CRRT. The patient maintained normal sinus rhythm on sotalol without adverse events. IV sotalol in the setting of ECMO and CRRT was safe and effective in controlling refractory hemodynamically compromising accelerated idioventricular rhythm unresponsive to amiodarone.

Bidirectional Glenn for residual outflow obstruction in Tetralogy of Fallot.

BACKGROUND: Residual right ventricular outflow obstruction during Tetralogy of Fallot repair necessitates peri-operative revision often requiring trans-annular patch with its negative sequels. Bidirectional Glenn shunt in this setting reduces trans-pulmonary gradient to avoid revision. METHODS: Bidirectional Glenn shunt was added during Tetralogy repair in patients with significant residual obstruction. A total of 53 patients between January, 2011 and June, 2018 were included. Final follow-up was conducted in July, 2018. RESULTS: Mean age at operation was 5.63±3.1 years. Right to left ventricular pressure ratio reduced significantly (0.91±0.09 versus 0.68±0.05; p<0.001) after bidirectional Glenn, avoiding revision in all cases. Glenn pressures at ICU admission decreased significantly by the time of ICU discharge (16.7±3.02 versus 13.5±2.19; p<0.001). Pleural drainage ≥ 7 days was seen in 14 (26.4%) patients. No side effects related to bidirectional Glenn-like facial swelling or veno-venous collaterals were noted. Mortality was 3.7%. Discharge echocardiography showed a mean trans-pulmonary gradient of 32.11±5.62 mmHg that decreased significantly to 25.64±5 (p<0.001) at the time of follow-up. Pulmonary insufficiency was none to mild in 45 (88.2%) and moderate in 6 (11.8%). Mean follow-up was 36.12±25.15 months (range 0.5-90). There was no interim intervention or death. At follow-up, all the patients were in NYHA functional class 1 with no increase in severity of pulmonary insufficiency. CONCLUSION: Supplementary bidirectional Glenn shunt significantly reduced residual right ventricular outflow obstruction during Tetralogy of Fallot repair avoiding revision with satisfactory early and mid-term results.