Severe Pulmonary Valve Regurgitation Research Articles

Results of Vertical Infra-Axillary Thoracotomy for Total Repair of Tetralogy of Fallot.

To demonstrate the efficacy of minimally invasive surgery via a vertical infra-axillary incision for complete tetralogy of Fallot (TOF) correction. In a study conducted from April to October 2023, 33 patients with TOF underwent total repair using this approach. On average, the patient age was 5.94 ± 2.68 months, weight was 6.49 ± 0.97 kg, and mean z-score index for the pulmonary valve annulus was -1.38 ± 0.92. Results also highlighted abnormal coronary artery pathways in 18.2% of cases, including 1 patient with dextrocardia and situs inversus. The average incision length was 4.01 ± 0.6 cm, with bypass and clamping times of 95.42 ± 33.19 min and 69.24 ± 28.15 min, respectively. Preservation of the pulmonary valve annulus was achieved in 67% of patients. No postoperative deaths occurred, and there were no significant ventilation differences between groups. After surgery, no severe pulmonary valve regurgitation was observed, with patients remaining in excellent condition throughout the 7-month follow-up. The pulmonary valve pressure gradient after the procedure was 23.97 ± 10.65 mm Hg, and no heart failure cases were reported per the Ross classification at the latest follow-up. The vertical infra-axillary incision approach for total TOF repair is safe, effective, and cosmetically advantageous.

A CASE OF RIGHT HEART FAILURE: WHEN IT IS NOT PULMONARY EMBOLISM

Abstract A 66–year–old patient, showing exertional dyspnea and atypical chest pain, came to our attention with instrumental findings at echocardiogram of right ventricular failure and was hospitalized due to worsening signs and symptoms of right heart failure. Signs of right ventricular strain and the McGinn–White sign (S1Q3T3 pattern) were seen on the ECG, leading to the suspicion of pulmonary thromboembolism (PE). Pulmonary CT angiography confirmed bilateral PE involving the main pulmonary artery. Oral anticoagulant therapy was initiated with initial partial benefit and relief of symptoms. However, after four weeks, the patient was readmitted for severe heart failure and signs of low cardiac output. Echocardiography showed the presence of a conspicuous, mobile, isoechoic mass occupying most of the main pulmonary artery, suggesting thrombosis. The patient underwent urgent surgical thromboendoarterectomy extended to the third segmental left branches and to the second segmental right branches of the pulmonary artery; a severe pulmonary valve regurgitation was observed and therefore the valve was replaced with a Edwards–Magna pericardial bioprosthetic valve. ECMO (Extracorporeal Membrane Oxygenation) was initiated due to haemodinac instability. In the postoperative period, the severe heart failure worsened, unresponsive to pharmacological treatments and mechanical support, leading to the patient passing away in the following days. Unexpectedly, histological analysis of the surgical specimen revealed neoplastic cells with vascular/endothelial differentiation; fluorescence in situ hybridization (FISH) detected the MDM2 gene (cfr.23–F–00275), involved in the p53 protein regulation mechanisms, which is consistent with a diagnosis of angiosarcoma. Pulmonary–artery angiosarcomas are extremely rare (few anecdotical cases are described in literature) and resemble the CT–signs and clinical features of PE, seriously delaying the diagnosis. They are usually associated with a worse prognosis than PE, one and a half months survival time when inoperable. Radical surgery, associated with thirty six and a half months survival time, is often unfeasible because of locally advanced disease or metastatic disease already present at diagnosis. The main differences with PE are the more proximal and almost–occluding involvement of the pulmonary artery, the absence of peripheral vein thrombosis and the absence of response to anticoagulant therapy.

May 2024 Medical Image of the Month: Hereditary Hemorrhagic Telangiectasia in a Patient on Veno-Arterial Extra-Corporeal Membrane Oxygenation

No abstract available. Manuscript truncated after 150 words. A 54-year-old man with a complex cardiac history, including Tetralogy of Fallot requiring Blalock-Taussig shunt in infancy, infundibular patch repair at age 7, and bioprosthetic tricuspid valve replacement at age 52, had ongoing frequent hospitalizations with decompensated right ventricular heart failure secondary to native pulmonary valve mixed stenosis plus regurgitation and left pulmonary artery stenosis. His case was further complicated by his history of hereditary hemorrhagic telangiectasia (HHT) with recurrent epistaxis and recent GI bleeds with multiple angiodysplastic lesions throughout the stomach, duodenum, and descending colon which were previously treated with argon plasma coagulation. The patient was admitted to our hospital in NYHA class IV heart failure receiving a continuous dopamine infusion and aggressive diuresis. Upon admission, a right heart catheterization demonstrated severe pulmonary valve regurgitation, left pulmonary artery stenosis, and systemic hypoxemia suggestive of an intrapulmonary shunt. Admission transthoracic echocardiogram demonstrated normal left ventricular ejection fraction of 55-60%, a …

Left ventricle dysfunction in patients with critical neonatal pulmonary stenosis: echocardiographic predictors. A single-center retrospective study.

The aim of this study is to identify echocardiographic predictors of transient left ventricle dysfunction after pulmonary valve balloon dilatation (PVBD), in neonates with pulmonary valve stenosis (PVS) and atresia with intact septum (PAIVS) at birth. The study includes patients admitted at the Bambino Gesù Children Hospital from January 2012 to January 2017. Clinical, echocardiographic and cardiac catheterization data before and after PVBD were retrospectively analyzed. Twenty-nine infants were included in the study (21 male and eight female). The median age was 5.8 ± 7.1 days. Eight patients developed transient LV dysfunction (three PAIVS and five PVS) and comparing data before and after the procedure, there was no difference in right ventricle geometrical and functional parameters except for evidence of at least moderate pulmonary valve regurgitation after PVBD. Moderate to severe degree pulmonary valve regurgitation was significant associated to LV dysfunction (p < 0.05) in PVS and PAIVS patients.

Abstract 14229: Carcinoid Heart Disease: What Do We Know About Characteristics and Outcomes of Tricuspid Valve Replacement? A Nationwide Analysis

Introduction: In patients with severe tricuspid and pulmonary valve regurgitation and symptoms of heart failure secondary to carcinoid heart disease, valve replacement is the only effective treatment and can improve survival. Previous data on outcomes of valve replacement in carcinoid heart disease have been limited to case reports or single-centre study. Therefore we aimed to perform a nationwide database to ascertain the outcomes of tricuspid valve replacement (TVR) in carcinoid heart disease. Methodology: We utilized nationwide readmission database (NRD) to identify hospitalizations for tricuspid valve replacement in 2018. Hospitalizations with valve replacement for infective endocarditis were excluded. Characteristics and outcomes were compared between patients undergoing TVR for carcinoid versus other reasons. Multivariable logistic regression was performed to examine the predictors of in-hospital mortality after TVR. Results: A total of 1192 hospitalizations for TVR were included in the analysis of which 57 had carcinoid heart disease. There were no significant differences in short-term outcomes including in-hospital mortality (12.6% vs 11.5%, p=0.839), acute renal failure, blood transfusion, cardiogenic shock, new pacemaker insertion, length of stay and 30-day readmissions between TVR for carcinoid vs other etiologies (Table). The independent predictors of mortality in patients undergoing TVR were advanced liver disease (4.67 (2.59-8.43), p&lt;0.001), end stage renal disease (5.09 (2.10-12.01), p&lt;0.001), acute heart failure (2.00 (1.10-3.67), p=0.022), malnutrition (2.24 (1.13-4.31), p=0.017), and age (1.02 (1.01-1.04), p=0.020), whereas carcinoid heart disease was not an independent predictor of mortality (aOR: 1.52 (0.39-4.65), p=0.497). Conclusion: Tricuspid valve replacement in patients with carcinoid heart disease is associated with comparable short-term outcomes as for other etiologies.

Percutaneous Balloon Pulmonary Valvuloplasty of Critical Pulmonary Stenosis and severe pulmonary stenosis in Neonates and Early Infancy: A Challenge in the Cyanotic.

Introduction: Pulmonary stenosis with an intact ventricular septum (PS-IVS) is one of the common causes of cyanotic heart disease in neonates with diverse morphologies as well as management and treatment protocols. The aim of this study was to evaluate short and midterm results of balloon pulmonary valvuloplasty (BPV) for this disorder. Methods: Between 2012 and 2016, Totally 45 neonates and infants under 6 months old were evaluated.The patients had a minimum right-to-left ventricular pressure ratio of 1, right-to-left shunting at the patent foramen ovale or atrial septal defect level, and tricuspid valve Z-scores higher than -4. Results: Immediately after the procedure, the right ventricular pressure dropped to the normal values in 8 (20%) patients. The immediate procedural success rate was seen in 42 (93.3%) cases: the right-to-left ventricular pressure ratio dropped to below 50% or the level of O2 saturation rose above 75%. Of three cases unresponsive to BPV, two of them underwent patent ductus arteriosus (PDA) stenting and one procedural death occurred. At 6 months’ follow-up, of 42 patients, this pressure was still with in the normal range in 36 (80%) infants, while it had returned to high values in 9 (20%) patients and necessitated repeat valvuloplasty. After BPV, severe pulmonary valve regurgitation was observed in14.2% patients; the condition was more common when high-profile noncompliant balloons were used. Conclusion: Balloon pulmonary valvuloplasty in infants with PS-IVS confers acceptable results insofar as it improves echocardiographic parameters and hemodynamic changes at short- and midterm followups.Balloon selection with sizes more than 1.2 of the diameter of the pulmonary valve annulus and the use of noncompliant high-pressure balloons results in higher degrees of pulmonary regurgitation.

Abstract 13811: The Clinical Significance of End-diastolic Forward Flow on the Prognosis After Pulmonary Valve Replacement in Patients Late After Repair of Tetralogy of Fallot

Introduction: Restrictive right ventricular physiology (r-RVP) is common in late after repair of tetralogy of Fallot (TOF) and reported to reflect diastolic dysfunction. Right ventricular (RV) diastolic dysfunction sometimes remains after pulmonary valve replacement (PVR) and is associated with arrhythmia. Pulmonary arterial end-diastolic forward flow (EDFF) is considered as a marker of r-RVP, and associated with RV volume, right atrial (RA) function, and the degree of pulmonary valve regurgitation (PR). The aim of this study is to evaluate the impact of EDFF before PVR on the clinical outcomes after PVR in patients with repaired TOF. Methods: This was a single-center, retrospective review of 46 patients who underwent PVR for moderate to severe PR between 2003 and 2019. Cases were examined EDFF before PVR and divided into two groups: with EDFF (EDFF+, n=23) and without EDFF (EDFF-, n=23). Patients with histories of atrial tachyarrhythmia underwent concomitant maze procedure. RV and RA volume were evaluated by magnetic resonance imaging. Post-PVR survival and the development of arrhythmia were assessed. Results: Age at PVR was 38±14 in EDFF+ and 35±10 years old in EDFF- (p=0.41), and the incidence of preoperative arrhythmia was not different (30% and 35%, p=1.0). RVESVI (102±24 and 86±26 ml/m 2 , p=0.048) and RAVI (84±19 and 70±20 ml/m 2 , p=0.025) before PVR, and RVEDVI (116±27 and 100±24 ml/m 2 , p=0.04) and RVESVI (71±23 and 55±16 ml/m 2 , p=0.01) at one year after PVR were greater in EDFF+. One patient in each group died due to non-cardiac disease. 5-year atrial tachyarrhythmia free rate was 62% in EDFF+ and 100% in EDFF- (Log-rank p=0.004). Multivariate Cox regression analysis revealed EDFF before PVR was a risk factor for atrial tachyarrhythmia after PVR (Hazard ratio 17 (95% CI, 2.2-406), p=0.025). Conclusions: EDFF before PVR was a significant risk factor for the development of postoperative atrial tachyarrhythmia. EDFF can complement the current indication for PVR.

Early Outcome of Balloon Pulmonary Valvuloplasty for Pulmonary Valve Stenosis in Adolescents and Adults: Experience at A Tertiary Care Cardiac Institute

Background: Balloon pulmonary valvuloplasty is the procedure of choice for pulmonary valve stenosis in any age group. The data on efficacy of the procedure in adolescent and adult population is scanty in Pakistan. Objective: To determine the early outcome of balloon pulmonary valvuloplasty in adolescents and adults. Study Design: Descriptive retrospective case series. Settings: Pediatric cardiology department of Faisalabad Institute of Cardiology, Faisalabad-Pakistan. Duration: Three years and Six months from 01-01-2016 to 30-06-2019. Methodology: All consecutive patients of age 10 years or above who underwent percutaneous balloon pulmonary valvuloplasty in the study period were enrolled. The outcome parameters including successfulness and complications of procedure recorded.&nbsp; The procedure was labelled successful if peak to peak pressure gradient across pulmonary valve reduced to less than 50% or more of the pre procedure value, suboptimal if gradient reduced by 25-49% and unsuccessful if gradient reduced by less than 25%. Results: A total of 41 patients underwent balloon pulmonary valvuloplasty procedure. Mean age was 22.2± 10.5 years while female to male ratio was 1.05:1. Majority of the patients (n=26, 63.4%) were symptomatic and dyspnea on exertion was the dominant symptom (n=16, 39%). Majority of the patients had doming pulmonary valve (n=37, 90.2%). Mean balloon to pulmonary valve annulus ratio was 1.24 ± 0.1 (range 1.1-1.43). The procedure was successful in all patients (n=41, 100%) as the mean peak to peak pressure gradient across pulmonary valve decreased from 121.9 ± 53.2 mmHg (pre valvuloplasty) to 30.1±11.9 mmHg (post valvuloplasty) irrespective of doming or dysplastic valve. There were no major complications. Transient self-limiting vaso-vagal syncope was noted in 7.3% of patients (n=3). On post procedure echocardiography there was not a single case of moderate or severe pulmonary valve regurgitation (PR) while mild PR was observed in 48.8% patients (n=20). Conclusion: Balloon pulmonary valvuloplasty in adolescent and adult patients is a very safe procedure with high success but very low complications rate.

The constrictive consequences of pericardial calcifications.

A 53‐year‐old man presented with symptomatic severe pulmonary valve regurgitation. He underwent a diagnostic catheterization. A heavily calcified pericardium and the pressure tracings illustrate typical features of constrictive pericarditis physiology, including the "square root sign." This condition is important to recognize given the progressive nature and poor prognosis if untreated.

Right Ventricle Has Normal Myofilament Function But Shows Perturbations in the Expression of Extracellular Matrix Genes in Patients With Tetralogy of Fallot Undergoing Pulmonary Valve Replacement.

BackgroundPatients with repair of tetralogy of Fallot (rToF) who are approaching adulthood often exhibit pulmonary valve regurgitation, leading to right ventricle (RV) dilatation and dysfunction. The regurgitation can be corrected by pulmonary valve replacement (PVR), but the optimal surgical timing remains under debate, mainly because of the poorly understood nature of RV remodeling in patients with rToF. The goal of this study was to probe for pathologic molecular, cellular, and tissue changes in the myocardium of patients with rToF at the time of PVR.Methods and ResultsWe measured contractile function of permeabilized myocytes, collagen content of tissue samples, and the expression of mRNA and selected proteins in RV tissue samples from patients with rToF undergoing PVR for severe pulmonary valve regurgitation. The data were compared with nondiseased RV tissue from unused donor hearts. Contractile performance and passive stiffness of the myofilaments in permeabilized myocytes were similar in rToF‐PVR and RV donor samples, as was collagen content and cross‐linking. The patients with rToF undergoing PVR had enhanced mRNA expression of genes associated with connective tissue diseases and tissue remodeling, including the small leucine‐rich proteoglycans ASPN (asporin), LUM (lumican), and OGN (osteoglycin), although their protein levels were not significantly increased.ConclusionsRV myofilaments from patients with rToF undergoing PVR showed no functional impairment, but the changes in extracellular matrix gene expression may indicate the early stages of remodeling. Our study found no evidence of major damage at the cellular and tissue levels in the RV of patients with rToF who underwent PVR according to current clinical criteria.

Right ventricular dilatation in patients with pulmonary regurgitation after repair of tetralogy of Fallot: How fast does it progress?

Objective:Pulmonary valve regurgitation (PR) and right ventricular (RV) dilatation are important residual findings after surgical repair of tetralogy of Fallot (TOF). We sought to describe the natural course of RV dilatation over time in patients with severe PR after TOF repair and to determine risk factors for quick progression of RV dilatation and dysfunction.Methods:Data of 85 consecutive TOF patients with PR and RV dilatation, undergoing serial cardiovascular magnetic resonance (CMR) scans between July 2002 and December 2016 in two institutions, were retrospectively reviewed. The dataset was analyzed regarding right and left ventricular (LV) volume and function and potential risk factors of progressive RV dilatationResults:There was no significant increase in RV end-diastolic volumes (RVEDVi) indexed body surface area (BSA) (median 150 [81–249] vs. 150 [82–260] mL/m2) and end-systolic volumes indexed for BSA (RVESVi) (75 [20–186] vs. 76 [39–189] mL/m2) between the first and last CMR in the overall group. Similarly, there were no significant changes in LV volumes indexed for BSA (LVEDVi 78 [56–137] vs. 81 [57–128] mL/m2 and LV end-systolic volume index 34 [23–68] vs. 35 [18–61] mL/m2). Global function remained also unchanged for both ventricles. RVEDVi increased statistically significantly (≥20 mL/m2) in twenty patients (24%) from 154 mL/m2 (87–237) to 184 mL/m2 (128–260, P < 0.001). LV dimensions showed a similar trend with LVEDVi increase from 80 ml/m2 (57–98) to 85 ml/m2 (72–105, P = 0.002). Shorter time interval between repair and first CMR was the only risk factor predictive for progressive RV dilatation.Conclusion:In the majority of patients with repaired TOF and severe PR, RV dilatation is unchanged during a follow-up of 3 years. RV dilatation seems to progress early after surgery and subsequently stabilize. RV dilatation significantly progresses in a subgroup of 24% of patients, with a shorter time interval since surgical repair.

Comparing Two Methods for the Right Ventricle Evaluation in TOF Patients with Pulmonary Regurgitation Following Total Repair: Pulsed Doppler and Tissue Doppler Imaging

Background: The Tei index is used to evaluate the overall performance of the right ventricle. The index can be measured with pulsed Doppler echocardiography (PW) and tissue Doppler imaging (TDI). Some studies show that severe pulmonary valve regurgitation (PR) can influence the Tei index calculated by the PW method. Objectives: The purpose of this study was to compare PW and TDI in calculating the Tei index in PR patients following TOF reconstruction. Methods: We evaluated 21 patients with severe PI who were reconstructed and 21 age-matched normal children. All the children went through PW or TDI. The intensity of Sa, Ea, Aa, IVCT, and the Tei index were calculated. Results: The mean age of the patients and controls was 3.8 ± 3.2 and 3.7 ± 2.7 years, respectively. There was no difference in the Tei index between healthy children and TOF patients calculated by the PW method (0.30 ± 0.10 vs. 0.35 ± 0.06; P = 0.059). However, TOF patients had a lower intensity of Sa, Ea, and Aa than healthy children. Furthermore, the isovolumic interval of both IVCT and IVRT was longer in TOF patients than in healthy children (43.53 ± 8.82 vs. 25.77 ± 10.14 and 87.73 ± 10.91 vs. 68.36 ± 8.96, respectively; P = 0.001 for both) but the S duration had no difference between the groups (265.1 ± 18.1 in TOF patients vs. 266.8 ± 28.0 in controls). The Tei index calculated by TDI was significantly higher in TOF patients than in healthy children (0.50 ± 0.10 vs. 0.36 ± 0.06; P = 0.001). Moreover, the Tei index calculated by TDI was higher than the Tei index calculated by PW in TOF patients (0.50 ± 0.10 vs. 0.30 ± 0.10; P = 0.001). Conclusions: TDI is a more accurate method for calculating the Tei index for evaluating the overall performance of the right ventricle in PR patients after TOF reconstruction, and the Tei index calculated through PW had limitations for this purpose.

Evaluation using a four-dimensional imaging tool before and after pulmonary valve replacement in a patient with tetralogy of Fallot: a\xa0case report

BackgroundPulmonary regurgitation is a common complication after tetralogy of Fallot repair, resulting in right ventricular dysfunction, arrhythmia, and sudden death. However, the indications and optimal timing for pulmonary valve replacement are not fully known. We describe a case in which a four-dimensional imaging tool was useful in the decision to re-operate, thus resulting in decreased energy loss and improved right ventricular function after the re-operation for tetralogy of Fallot.Case presentationA 54-year-old Japanese woman visited our hospital due to palpitations and wide QRS tachycardia with persistent tiredness for several months. She underwent repair of tetralogy of Fallot when she was 2-years old. An electrocardiogram showed prolonged QRS duration (199 msec) with a complete right bundle branch block and an echocardiograph demonstrated that her right ventricle was highly enlarged and had poor contraction, and severe pulmonary valve regurgitation with one leaflet flail. Four-dimensional flow magnetic resonance imaging demonstrated that regurgitant volumes and regurgitant fractions of pulmonary regurgitation were calculated as 63.12 ml and 54.0%, respectively. Right ventricular end-diastolic/end-systolic volume index was 169.54/99.76 mL/m2, and the cardiac index was 1.78 L/minute per m2. Flow energy loss was 2.93 mW, which is estimated to be three times higher than normal controls. An electrophysiological study showed an intact anterior internodal pathway and a slow pathway just through the outside of the right atriotomy line scar, which is supposed to cause a re-entry circuit. We decided to perform a pulmonary valve replacement and a right maze procedure. A 27 mm bioprosthetic valve was implanted in the native pulmonary annulus with a supra-annular position. Concomitantly, the right maze procedure was performed. A four-dimensional flow magnetic resonance imaging done 3 months later showed that right ventricular end-diastolic/end-systolic volume index had significantly reduced to 85.24/55.41 mL/m2 and the cardiac index had increased from 1.78 to 2.58 L/minute per m2. Energy loss had greatly improved from 2.93 to 1.48 mW.ConclusionsA four-dimensional imaging tool was useful in the decision to re-operate, thus resulting in decreased energy loss and improved right ventricular function after the re-operation for tetralogy of Fallot.

Surgical Repair of Tetralogy of Fallot With Absent Pulmonary Valve: Favorable Long-Term Results

Tetralogy of Fallot with absent pulmonary valve syndrome (ToF-APVS) is a rare variant of tetralogy, associated with severe pulmonary valve regurgitation and aneurysmal dilation of the pulmonary arteries (PAs). Reported outcomes after surgical corrections are limited to single center, older series and might not reflect the current outcome. We aim to use data from a national registry to evaluate short- and long-term outcomes after surgical repair of ToF-APVS, to serve for counselling and planning. All children undergoing ToF-APVS repair in the UK between 2002 and 2013 were included. Survival and freedom from reintervention were estimated using the Kaplan-Meier method, and univariable analysis was done using the Weibull regression model. A total of 98 children, 45% male, 10% with DiGeorge syndrome, median age of 213 days (1 day to 13 years) were included. Mortality at 30 days was 3.3%, higher for neonates (6.7% vs 2.7%, P = 0.4) and those on preoperative mechanical ventilatory support (16.7% vs 1.3%, P = 0.04). Survival was 92.1% and freedom from pulmonary valve or conduit replacement (PVR) 73.2% at 10 years. Neonates had worse survival (hazard ratio [HR] 6.2, P = 0.02), freedom from PVR (HR 4.5, P = 0.01), freedom from PAs arterioplasty (HR 6.6, P = 0.001), and overall freedom from any reintervention (HR 5.3, P < 0.001). Low weight at repair was associated with worse freedom from PVR (P = 0.02) and from PAs arterioplasty (P = 0.009), preoperative ventilatory support with increased mortality (P = 0.009), the presence of DiGeorge syndrome was associated with worse freedom from PVR (HR 4, P = 0.02). Surgical repair of ToF-APVS can be performed with low early and late mortality, with improving results even in those with preoperative mechanical ventilatory support. The need for right ventricular outflow tract reintervention, including on the PAs, is an expected issue in the long term for the majority of patients.

Impact of additional tricuspid valve annuloplasty in TOF patients undergoing pulmonary valve replacement.

Many patients with tetralogy of Fallot (TOF) who underwent surgical correction of their congenital cardiac malformation during infancy develop right ventricular dysfunction and exercise intolerance in the long term. The right ventricle (RV) dilates due to the development of severe pulmonary regurgitation (and secondary tricuspid insufficiency). To reduce RV dilation and improve exercise tolerance pulmonary valve replacement (PVR) is the common therapeutic strategy. Whether concomitant tricuspid valve repair (TVR) is beneficial in these pure volume-overload conditions is still unknown. Twenty-eight adults who received surgical PVR were included in the study and perioperative data including operative records, postoperative course and echocardiography before and after surgery was analyzed retrospectively. Mean age of the patients was 41.1±13.5 years and PVR with Carpentier Edwards Perimount bioprostheses was performed 32.0±9.5 years after initial cardiac surgery. Preoperative echocardiography revealed moderate RV dilation in 60.7% of the patients and severe pulmonary valve regurgitation in 82.3%. Ten patients underwent additional TVR. Indication was purely based on annular dilation (>40 mm), independent from the degree of tricuspid insufficiency. Periprocedural data of both groups did not differ significantly. However, ICU stay was significantly increased in patients who underwent additional TVR (P=0.0420) and these patients developed more complications (P=0.0407) while postoperative echography showed the same recovery of the RV function and diameters, independent of concomitant TVR. In grown-ups with congenital heart disease surgical PVR with or without TVR is a safe procedure with good short-term outcomes. As the RV remodels after volume reduction, indication for concomitant TVR should be restrictive.

Pulmonary valve replacement after repaired Tetralogy of Fallot.

In this review article, we describe pulmonary valve replacement (PVR) late after repaired Tetralogy of Fallot (TOF). Since the introduction of surgical intervention for patients with TOF in 1945, surgical management of TOF has dramatically improved early survival with mortality rates, less than 2-3%. However, the majority of these patients continue to experience residual right ventricular outflow tract pathology, most commonly pulmonary valve regurgitation (PR). The patients are generally asymptomatic during childhood and adolescence and, however, are at risk for severe PR later which can result in exercise intolerance, heart failure, arrhythmias, and sudden death. While it has been shown that PVR improves symptoms and functional status in these patients, the optimal timing and indications for PVR after repaired TOF are still debated. This article reviews the current state of management for the patient with PR after repaired TOF.

CYANOSIS AS A LONG TERM COMPLICATION OF PULMONARY VALVE SURGICAL VALVOTOMY

Severe pulmonary valve (PV) regurgitation can result from surgical or balloon valvotomy. If left untreated PV regurgitation in the setting of right ventricular non-compliance may lead to progressive cyanosis, increased morbidity and mortality. A 60-year-old white male with history of hypoxia

Tetralogy of Fallot repair in patients presenting after Infancy: A single surgeon experience.

Objective:To determine the early surgical outcomes of Tetralogy of Fallot (TOF) repair in children and young adults operated after the age of one year.Methods:In this retrospective study, 307 cases of primary repair of Tetralogy of Fallot were done between September 2012 to February 2017, at CPE Institute of cardiology, Multan. Out of 307 operated patients, 4 (1.3%) patients had previous modified Blalock Taussig shunts, 2 (0.6%) associated ASD with TOF, 3 (0.9%) co-association of TOF with PDA, 2 (0.6%) had large conal arterial branch crossing the annulus, 3 (0.9%) had dextrocardia with situs inversus, 12 (3.9%) TOF with double outlet right ventricle (DORV), 2 (0.6%) were associated with complete AV canal defect, 8 (2.60%) with absent pulmonary valve syndrome, 15 (5.5%) with left pulmonary artery stenosis. Data of post-operative complications and operative parameters was recorded for all patients.Results:Mean age of operated patients was 9.56±4.89 years. Post-operative complications occurred in 7.8% of patients. Most common post-operative complications were pleural effusion with a frequency of 12(3.9%) patients, and complete heart block in one patient. Insignificant small residual VSD was diagnosed in 8 (2.6%) patients. One moderately large VSD was closed surgically after one year of 1st surgery. Moderate to severe pulmonary valve regurgitation was diagnosed in 114 (37.1%) patients. Mild to moderate tricuspid regurgitation in 15 (4.8%) patients and moderate right ventricular outflow tract obstruction (RVOT) in 16 (5.2%) patients. Thirty-day mortality was only four (1.3%).Conclusion:Surgical correction of Tetralogy of Fallot (TOF) in children after one year carries good operative outcomes with minimum morbidity and mortality.

Long term follow-up after the Ross procedure (RCD code: IV-5A.O)

Aims: The aim of this study was to analyze the incidence of long-term complications observed during follow-up of patients who had undergone the Ross procedure in childhood. Methods: The study engaged a cohort of 9 patients, all of whom were between 19 to 32 years old. Patients had been in the care of the Centre for Rare Cardiovascular Diseases since the age of 18. Clinical and echocardiographic data were collected from the follow-up period. Results: 78% of patients had preserved global systolic function of the left ventricle, and 56% had dilatation of the ascending aorta. Due to the aneurysm of the ascending aorta one patient required the Bentall de Bono procedure. Another patient underwent a reoperation because of endocarditis of the pulmonary homograft with severe aortic and pulmonary regurgitation. 1/3 of the studied patients were being considered for a reoperation. 23% of patients developed severe pulmonary valve regurgitation, 33% moderate. 67% of patients developed mild to moderate pulmonary valve stenosis. Most patients were in NYHA class I-II. Conclusion: Late complications are frequent in this group of patients, and hence they require surveillance in specialized Centers for Grown-up Congenital Heart Diseases. JRCD 2016; 3 (1): 9–13

Early- and mid-term results of off-pump pulmonary valve implantation: a single-center experience

Background: This study aims to assess the early- and midterm results of off-pump pulmonary valve implantation (PVI). Methods: Between October 2006 and February 2012, nine patients (5 males, 4 females; mean age 18.4±6.3 years; range 8 to 27 years) underwent off-pump PVI with an injectable stent. Echocardiography revealed severe pulmonary valve regurgitation, progressive right ventricular dilatation and dysfunction in all patients. No residual ventricular septal defect (VSD) was seen. Results: We implanted a 23 mm valve in one patient, a 25 mm valve in two patients, a 27 mm valve in two patients, a 29 mm valve in three patients and a 31 mm valve in one patient. Postoperative echocardiographic examination revealed no regurgitation in seven patients with mild valve regurgitation in two patients. The mean peak systolic gradient was 12.6±3.7 mmHg, while the mean systolic gradient was 8.3±2.4 mmHg. The mean follow-up was 34±17 months (range, 1.5 to 58 months). None of the patients needed reoperation. Conclusion: The advantages of off-pump PVI include the applicability without cardiopulmonary bypass, availability of large-sized valves, the absence of any interventional limitation and the opportunity for fixation into the pulmonary artery to avoid valve migration. No-react nature of the valve also minimizes the degeneration risk of the valve.