Severe Pulmonary Regurgitation Research Articles

Discordance between 2D and 4D flow in the assessment of pulmonary regurgitation severity: a right ventricular remodeling follow-up study.

Pulmonary regurgitation (PR) is common in adult congenital heart disease (ACHD). 2D phase contrast MRI is the reference method for the quantification of PR and helps in the decision of pulmonary valve replacement (PVR). 4D flow MRI can be an alternative method to estimate PR but more validation is still needed. Our purpose was to compare 2D and 4D flow in PR quantification using the degree of right ventricular remodeling after PVR as the reference standard. In 30 adult patients with a pulmonary valve disease recruited between 2015 and 2018, PR was assessed using both 2D and 4D flow. Based on the clinical standard of care, 22 underwent PVR. The pre PVR estimate of PR was compared using the post-operative decrease in right ventricle end-diastolic volume on follow-up exam as reference. In the overall cohort, regurgitant volume (Rvol) and regurgitant fraction (RF) of PR measured by 2D and 4D flow were well correlated but with moderate agreement in the overall cohort (r = 0.90, mean diff. -14 ± 12.5mL; and r = 0.72, mean diff. -15 ± 13%; all p < 0.0001). Correlations between Rvol estimates and right ventricle end-diastolic volume decrease after PVR was higher with 4D flow (r = 0.80, p < 0.0001) than with 2D flow (r = 0.72, p < 0.0001). In ACHD, PR quantification from 4D flow better predicts post-PVR right ventricle remodeling than that from 2D flow. Further studies are needed to evaluate the added value of this 4D flow quantification for guiding replacement decision. • Using 4D flow MRI allows a better quantification of pulmonary regurgitation in adult congenital heart disease than 2D flow when taking right ventricle remodeling after pulmonary valve replacement as a reference. • A plane positioned perpendicular to the ejected flow volume as allowed by 4D flow provides better results to estimate pulmonary regurgitation.

Characterization of Favorable Right Ventricular Dimensions for Optimal Reverse Remodeling Following Pulmonary Valve Replacement

We sought to couple current cardiac magnetic resonance (CMR) thresholds of right ventricular (RV) size and function with longitudinal trajectories of RV recovery, after pulmonary valve replacement (PVR). We aimed to identify optimal timing of PVR and couple CMR-based metrics with contemporaneous echocardiographic metrics. From June 2002 to January 2019, 174 patients with severe pulmonary regurgitation and peak RV outflow tract gradient <30 mm Hg underwent PVR at Cleveland Clinic. Mean age was 35 ± 16 years and 60 (34%) had concomitant tricuspid valve surgery. RV end diastolic area index (RVEDAi) and function metrics were measured by offline image review on preoperative and 794 postoperative echocardiograms. Contemporaneous RV end diastolic volume index (RVEDVi) was assessed on CMR and correlated to RVEDAi. Multiphase nonlinear mixed-effects models were used to analyze the longitudinal change in RV size and function after PVR. RVEDAi was correlated with RVEDVi (P < 0.0001, r = 0.59). RVEDAi decreased slowly over 10 years following PVR. An inflection point at 24 cm2/m2 was noted at 1 year post-PVR and was associated with failure of RV reverse remodeling and RVEDVi ≥150 mL/m2. Compared to patients with preoperative RVEDVi ≥150 mL/m2, patients with RVEDVi <150 mL/m2 had accelerated recovery of longitudinal trajectories of RV size and function metrics on echocardiograms. Reverse remodeling of RV following PVR is an ongoing process. Current accepted threshold values for PVR are associated with greatest RV recovery, suggesting that earlier PVR is warranted. Echocardiography can potentially be utilized in lieu of CMR for surveillance and interventional triage.

Visibility of Pulmonary Valve and Pulmonary Regurgitation on Intracardiac Echocardiography in Adult Patients with Tetralogy of Fallot.

Pulmonary regurgitation (PR) is a risk factor for sudden cardiac death in adult patients with repaired tetralogy of Fallot (TOF). However, transthoracic echocardiography (TTE) cannot fully visualize the pulmonary valve (PV) and PR. We investigated whether intracardiac echocardiography (ICE) could visualize the PV and PR better than TTE. Thirty adult patients with TOF (mean age 33 ± 15 years) scheduled for cardiac catheterization underwent ICE. The visualization of PV and the severity of PR were classified into three grades. ICE depicted the PV better than TTE (ICE vs. TTE: not visualized, partially visualized, and fully visualized: n = 1 [3%], n = 13 [43%], and n = 16 [53%] vs. n = 14 [47%], n = 13 [43%], and n = 3 [10%], p &lt; 0.001). Especially in patients after pulmonary valve replacement (PVR), the PV was more fully visualized by ICE. The assessment of PR by TTE underestimated the severity of PR in comparison to cardiac magnetic resonance imaging (MRI) (severe PR: 8 [28%] vs. 22 [76%], p = 0.004), while there was no discrepancy between the results of ICE and MRI (21 [72%] vs. 22 [76%], p = 1.000). In comparison to TTE, ICE can safely provide better visualization of the PV and PR in adults with TOF, especially in patients who have undergone PVR.

Outcomes of Self-Expanding Transcatheter Pulmonary Valves: Extended Follow-Up of a Prospective Trial

<b>Background:</b> The Venus-P valve was the first self-expanding valve used world-wide for transcatheter pulmonary valve replacement (TPVR) in patients with severe pulmonary regurgitation (PR). We intended to report the extended follow-up results from the prospective trial (No. NCT02590679). <b>Methods:</b> A total of 38 patients with severe PR (mean age 24.2 ± 13.2) were included. Follow-up data were obtained after implanted at 1, 6, and 12 months and yearly after. The frame geometry was assessed on post-implant computer tomography (CT) scanning by calculating the non-circularity [circularity ratio (minimum diameter/maximum diameter) < 0.9] and under-expansion [expansion ratio (derived external valve area/nominal external valve area) < 0.9). Adverse events (all-cause mortality, reintervention, valve dysfunction, stent fracture and endocarditis) were recorded. <b>Results:</b> All valves were implanted successfully with normal function at discharge. Geometric CT analysis showed under-expanded valve was detected in 22 patients (63%) and non-circular valve was seen in 16 patients (46%). During a median follow-up of 4.8 years (range 0.3–8.1), there were 1 death and 1 surgical explant, both resulting from endocarditis. Five-year freedom from valve dysfunction and stent fracture were 84.8% (95%CI 74.8–94.7) and 83.5% (95%CI 73.8–93.2). Endocarditis occurred in 3 patients at a median time of 7 months. Stent fracture was more common in patients with non-circularity stents. <b>Conclusion:</b> TPVR using Venus-P valve is associated with favorable outcomes at 5 years. Non-circular shapes in the valve level may have a higher risk of stent fracture.

Impact of Pulmonary Arterial Elastance on Right Ventricular Mechanics and Exercise Capacity in Repaired Tetralogy of Fallot.

Pathophysiological changes of right ventricle (RV) after repair of tetralogy of Fallot (TOF) are coupled with a highly compliant low-pressure pulmonary artery (PA) system. This study aimed to determine whether pulmonary vascular function was associated with RV parameters and exercise capacity, and its impact on RV remodeling after pulmonary valve replacement. In a total of 48 patients over 18 years of age with repaired TOF, pulmonary arterial elastance (Ea), RV volume data, and RV-PA coupling ratio were calculated and analyzed in relation to exercise capacity. Patients with a low Ea showed a more severe pulmonary regurgitation volume index, greater RV end-diastolic volume index, and greater effective RV stroke volume (p=0.039, p=0.013, and p=0.011, respectively). Patients with a high Ea had lower exercise capacity than those with a low Ea (peak oxygen consumption [peak VO2] rate: 25.8±7.7 vs. 34.3±5.5 mL/kg/min, respectively, p=0.003), while peak VO2 was inversely correlated with Ea and mean PA pressure (p=0.004 and p=0.004, respectively). In the univariate analysis, a higher preoperative RV end-diastolic volume index and RV end-systolic volume index, left ventricular end-systolic volume index, and higher RV-PA coupling ratio were risk factors for suboptimal outcomes. Preoperative RV volume and RV-PA coupling ratio reflecting the adaptive PA system response are important factors in optimal postoperative results. We found that PA vascular dysfunction, presenting as elevated Ea in TOF, may contribute to exercise intolerance. However, Ea was inversely correlated with pulmonary regurgitation (PR) severity, which may prevent PR, RV dilatation, and left ventricular dilatation in the absence of significant pulmonary stenosis.

Early postoperative tachyarrhythmias in adult congenital heart surgery: an eight-year review at a tertiary university hospital in Thailand.

Tachyarrhythmias are a common and significant complication following surgery for adult congenital heart disease (CHD), adversely affecting morbidity. This study aimed to assess the incidence, characteristics, and risk factors of early postoperative tachyarrhythmias in adults undergoing CHD surgery. We conducted a retrospective cohort study at Siriraj Hospital, Bangkok, Thailand, including 311 adult patients who underwent elective CHD surgeries from March 2013 to November 2020. We analyzed demographic and surgical data, focusing on new-onset tachyarrhythmias requiring interventions such as antiarrhythmic drugs or cardioversion. The patient cohort had a median age of 34 years (range, 18-78 years), with 34.7% male, predominantly diagnosed with atrial and ventricular septal defects (54.7%), and severe pulmonary regurgitation post-tetralogy of Fallot repair (22.2%). Postoperative tachyarrhythmias occurred in 12.9% of patients, with atrial fibrillation (AF; 67.5%) and atrial flutter (AFL; 14.5%) being the most frequent. Independent risk factors for tachyarrhythmias included older age [adjusted odds ratio (OR) =1.26 per 5-year increment; 95% confidence interval (CI): 1.12-1.42; P<0.001] and left atrial enlargement (adjusted OR=2.78; 95% CI: 1.31-5.85; P=0.007). Early postoperative tachyarrhythmias are prevalent in adults undergoing CHD surgery, with advanced age and left atrial enlargement as significant risk factors. These findings underscore the importance of early detection and management of septal defects and advocate for enhanced screening and healthcare strategies to reduce postoperative complications in this population.

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.

Right ventricular echocardiographic remodeling after pulmonary valve replacement in repaired Tetralogy of Fallot

BackgroundMany patients with repaired tetralogy of Fallot require reoperation in the medium to long-term for residual pulmonary valve regurgitation. Best timing for pulmonary valve replacement remains controversial. A balance needs to be found between protecting the patients from permanent right ventricular damage and insertion of a prosthetic valve with its inherent issues. In the current study we sought to investigate the right and left ventricular functional recovery following valve replacement in our tertiary care institution. ResultsIn a retrospective cross-sectional study patients with history of total correction of tetralogy of Fallot who had undergone pulmonary valve replacement due to severe pulmonary regurgitation between 2003–2018 were evaluated for post intervention right and left ventricular functional recovery. Clinical and full echocardiographic data before and after the surgery were recorded and compared. There was statistically significant improvement in RV size and function post pulmonary valve replacement. There was no statistically significant improvement in left ventricular systolic function. Twenty percent of patient had persisting severe right ventricular enlargement at least twelve months post-surgery. No patient had fully normalized right ventricular size and function in follow-up. ConclusionsPulmonary valve replacement leads to improvement in right ventricular size and function in patients with repaired tetralogy of Fallot. However normalization of functional parameters did not occur and the majority of the patients have residual right and left ventricular dysfunction following redo valve replacement surgery.

Early results of Pulsta® transcatheter heart valve in patients with enlarged right ventricular outflow tract and severe pulmonary regurgitation due to transannular patch.

The purpose of this study is to assess the feasibility, effectivity, and safety of a novel self-expandable valve system, Pulsta® transcatheter heart valve in patients with tetralogy of fallot and severe pulmonary regurgitation after transannular patch repair. Severe pulmonary regurgitation after tetralogy of fallot repair is a life-threatening problem and should be treated by pulmonary valve implantation. Although percutaneous pulmonary valve implantation has been ever increasingly used for this purpose, available balloon-expandable valves have limitations and cannot be used by most patients. Pulsta® transcatheter heart valve is a new self-expandable valve system and offers a new solution to be used in patients with different types of native right ventricular outflow tract geometry. Ten patients with severe regurgitation after tetralogy of fallot repair with a transannular patch have been enrolled in the study according to echocardiographic examination. MRI was used in asymptomatic patients to delineate the indication and the right ventricular outflow tract geometry. Pulsta® transcatheter heart valve implantation was performed in ten patients, and preprocedural, procedure, and 6 months follow-up findings of the patients were evaluated. Pulsta® pulmonary valve implantation was performed in ten patients successfully without any severe complications. Valve functions were perfect in six of ten patients, while the others had insignificant regurgitation by echocardiographic examination at the end of 6 months follow-up. This study showed that Pulsta® transcatheter heart valve is a feasible, effective, and safe method in the treatment of severe pulmonary regurgitation due to transannular patch repair in patients with tetralogy of fallot.

Abstract 11712: Transcatheter Reconstruction of Right Ventricular Outflow Tract After Severe External Compression by a Pseudoaneurysm in an Adult With Tetralogy of Fallot

Introduction: Increased survival of adults with tetralogy of Fallot (TOF) has led to the recognition of unanticipated complications. A rare complication after TOF repair is a right ventricle pseudoaneurysm (RV PSA). Often asymptomatic and incidentally found on imaging, the few reports of clinically significant RV PSA were mostly treated surgically. We present an adult with RV PSA after TOF repair treated by a novel transcatheter approach. Case Presentation: A 41 year old male with TOF and a valved right ventricle to pulmonary artery conduit presented with right ventricular failure due to right ventricular outflow tract obstruction (RVOTO) from an anterior RV PSA compressing the conduit (Figure 1). His case was further complicated by HFrEF, severe pulmonary regurgitation, and refractory atrial fibrillation requiring anticoagulation. Surgical intervention was not offered due to five prior sternotomies; he was not a transplant candidate due to substance abuse. CT imaging was used to create a virtual 3D model for interventional planning (Figure 1). A Covered CP Stent™ (B. Braun, Bethlehem, PA) on top of a Palmaz™ XL 5010 stent (Cordis Corp, Miami Lakes, FL) was anchored in the pulmonary valve ring and extended proximally to seal the PSA leak in the RVOTO, followed by Sapien S3 valve (Edwards, Irvine, CA) implantation in the proximal end of the stents (Figure 2). Right ventricular systolic pressure acutely decreased with improved systolic function. Conclusion: Adults with congenital heart disease often require unique approaches to intervention for managing the unnatural history of their conditions. 3D modeling of complex structures is a vital tool to plan these interventions.

Abstract 11155: Recovery of Tricuspid Valve Function After Resection of a Primary Ovarian Carcinoid Tumor

Case Presentation: A 51-year-old woman presented with a &gt;1-year history of bilateral leg edema, diarrhea, and facial flushing. Examination revealed a large abdominal mass, which CT scan confirmed to be a 15x11x13 cm ovarian mass with solid and cystic components. She underwent hysterectomy and bilateral salpingo-oophorectomy. Histopathology revealed a 17 cm ovarian dermoid cyst containing an insular carcinoid tumor. Her post-operative urinary 5-hydroxyindoleacetate (HIAA) level was 23 umol/d (normal 10-40 umol/d) and chromogranin-A level was 95 ug/L (normal &lt;94 ug/L), but positron emission tomography (PET) scan showed lymph node metastasis, prompting initiation of lanreotide, a somatostatin analogue. A perioperative echocardiogram revealed thickened, fixed, and retracted tricuspid and pulmonic valves with severe tricuspid and pulmonary regurgitation consistent with carcinoid heart disease. The right ventricle was dilated but showed preserved systolic function. Valve intervention was considered but deferred as right heart catheterization 15 weeks after surgery showed normal right-sided pressures. A follow-up echo 8 months post resection showed unchanged pulmonary regurgitation but significant improvement in tricuspid valve mobility with a corresponding reduction in tricuspid regurgitation to a mild-to-moderate level. Discussion: Thickened and fixed tricuspid valve leaflets with significant regurgitation represents a hallmark of carcinoid heart disease that can lead to progressive right heart failure, often necessitating surgical intervention. This is a rare case of carcinoid heart disease where there was marked improvement in tricuspid valve function after surgical resection of a primary carcinoid tumor and initiation of somatostatin analog therapy. It highlights the potential for timely treatment to reverse the deleterious effects of carcinoid tumors on cardiac valves, which may ultimately spare the need for cardiac surgery.

Abstract 10262: High Output Cardiac Failure Due to Kratom

Introduction: Kratom use has been known to the natives of Malaysia to increase energy and to treat pain. Over the recent past there has been an increase in Kratom usage in the U.S. with the ease of access. Case Summary: A 24-year-old male presented to the emergency department due to progressive shortness of breath and pedal edema over one month. The patient reported a history of daily Kratom use for the past 2 years. His vitals upon presentation were BP: 90/50 mmHg, HR 101 bpm, O2 saturation 100% and was afebrile. Relevant abnormal labs were: sodium 114 mEq, and a BNP of 2,747 pg/mL. Other labs including TSH and urine drug screen were unremarkable. Initial ECG showed normal sinus rhythm, right axis deviation and QTc of 552 ms. Chest X ray revealed a prominent pulmonary conus. After being admitted to the ICU, he had an appropriate gradual increase in sodium level over the next 4 days. The transthoracic echocardiogram showed an LVEF of 70-75% and multiple findings suggestive of severe pulmonary hypertension and severe tricuspid regurgitation (Fig. 1 parts A and B). His estimated PASP was 95 mm Hg. Right heart catheterization demonstrated the following pressures (in mmHg): RA 5/6, PA 52/26 (37), mean PCWP 7. Cardiac output and index were 8.9 L/min and 5 L/min/m2 by Fick, 7.7 L/min and 4.3 L/min/m2 by thermodilution. Four weeks after cessation of Kratom use he underwent a transthoracic echocardiogram which showed complete normalization of abnormal findings shown in Fig. 1 parts C and D. His RVSP normalized to 26 mm Hg. Conclusion: Previously reported adverse cardiovascular events from Kratom use include tachycardia, hypertension, chest pain, conduction defects, QTc prolongation as well as cardiac arrest. We present previously unknown complications of Kratom use, namely combined pre and post capillary pulmonary hypertension and high output heart failure.

Long-term outcome of repaired tetralogy of Fallot: Survival, tachyarrhythmia, and impact of pulmonary valve replacement.

Pulmonary valve replacement (PVR) is recommended for severe pulmonary regurgitation in repaired tetralogy of Fallot (rTOF). The purpose of this study was to investigate the event rate and effectiveness of PVR. A retrospective study of tetralogy of Fallot patients who survived total repair from 1970 to 2020 was conducted. We identified 1744 rTOF patients; 86.6% with classic rTOF, 11.5% with pulmonary atresia, 0.8% with endocardial cushion defect, and 1.1% with absent pulmonary valve. Annual risks of tachyarrhythmia/sudden cardiac arrest (SCA) increased to 0.295% and 1.338% in patients aged 10-30 and 30-60 years, respectively, without sex predominance. PVR (223 surgical and 39 percutaneous) event rate was 34.7% ± 2.1% by 30 years after repair (annual risk: 1.57% between 10 and 30 years after repair). The second PVR rate was 9.9% ± 4.1% by 20 years after the first PVR. Tachyarrhythmia/SCA risk was higher in PVR patients than in No PVR patients and was reduced in PVR patients without tachyarrhythmia/SCA before PVR. However, survival in patients with ventricular tachyarrhythmia/SCA still was better after PVR. At PVR, 13% of patients had tachyarrhythmia/SCA, which was the major predictor of events after PVR. Before PVR, although the ventricular tachyarrhythmia/SCA risks included QRS duration >160 ms and New York Heart Association functional class III or IV, supraventricular tachyarrhythmia was associated with PVR age ≥28 years and N-terminal pro-brain natriuretic peptide >450 pg/mL. Tachyarrhythmia/SCA occurrence and the need for PVR increased with age during young adulthood. PVR reduced subsequent arrhythmias only in those patients without arrhythmias before PVR.

Middle to long-term outcomes of surgical repair for atrioventricular septal defect: a single-center study.

The exact incidence and predictors of mortality and left atrioventricular valve (LAVV) re-operation in congenital atrioventricular septal defect (AVSD) repair are still unclear. This study analyzed the middle to long-term outcomes of surgical repair for AVSD. A total of 150 patients (69 males and 81 females) who underwent AVSD repair at Children's Hospital of Fudan University from January 2013 to December 2021 were divided into complete defect group (C-group, 67 cases), transitional defect group (T-group, 26 cases), and partial defect group (P-group, 57 cases). Outcomes during the peri-operative and 10-year follow-up periods were evaluated. The total mortality was 5.33% (8/150), including seven early deaths (10.4%) and no late deaths in the C-group, no early deaths (0%) and one late death (1.8%) in the P-group, and no early or late deaths in the T-group. Up to the last follow-up, severe LAVV regurgitation had occurred in 27 patients, including 16 in the C-group, four in the T-group, and seven in the P-group. In total, 12 (12/150, 8.0%) patients received LAVV re-operation, including seven in the C-group, three in the T-group, and two in the P-group. Cox regression analysis showed that pre-operative severe pulmonary hypertension (P=0.006) and severe LAVV regurgitation within 24 hours after the first surgery (P=0.023) were independent risk factors for mortality. ≥ Moderate LAVV regurgitation within the first 24 hours after surgery (P=0.014) was an independent risk factor for LAVV re-operation. Complete AVSD repair increased the risk of early death, severe LAVV regurgitation and re-operation. Pre-operative severe pulmonary hypertension and residual severe LAVV regurgitation indicated high risk for mortality. ≥ Moderate LAVV regurgitation within 24 hours after the first surgery predicted a high probability of LAVV re-operation.

Feasibility and Surgical Effect of Annulus Sparing in Consecutive Patients with Tetralogy of Fallot: A Retrospective Cohort Study

Objective: Annulus-sparing (AS) repair for tetralogy of Fallot (TOF) with a dysplastic pulmonary valve annulus (PVA) is a challenging procedure and is controversial. This study aimed to assess the feasibility and surgical effect of AS repair versus transannular patch enlargement (TAPE) repair, especially in individuals with dysplastic pulmonary valves. Methods: This retrospective cohort study included 375 pediatric patients with a primary diagnosis of TOF in the Center for Pediatric Cardiac Surgery of Fuwai hospital from January 2014 to June 2017. Among them, 60 consecutive and nonselective patients underwent 1-stage repair of TOF with aggressive PVA-preserving strategies performed by a single surgeon were enrolled in AS cohort. In AS cohort, patients were divided into AS, PVA z-score ≥−2 group (33 patients) and AS, PVA z-score &lt;−2 group (27 patients). During the same period, 315 patients underwent TAPE repair by other surgeons were enrolled as TAPE cohort, of these, 87 patients with PVA z-score ≥−2 were excluded. From the 228 patients in the TAPE group, 27 cases were selected as TAPE, PVA z-score &lt;−2 group according to the propensity score and 1:1 ratio with AS, PVA z-score &lt;−2 group. The primary outcome was a composite of reintervention, significant pulmonary regurgitation, and significant annular peak gradient (APG). Kaplan-Meier curve was plotted to show the survival rate of severe pulmonary regurgitation. Results: One death occurred after the TAPE operation in TAPE group, and 1 patient in the AS z ≥−2 group needed reintervention with a balloon. After a median follow-up of (30.3 ± 11.6) months, compared with AS z ≥−2 group, there was no difference in the technical performance score for severe pulmonary stenosis (APGs &gt; 20 mmHg) in the AS z &lt;−2 group. Compared with TAPE, AS repair was often accompanied by a postoperative APGs over 20 mmHg (P = 0.001). More patients underwent TAPE suffered from moderate or severe pulmonary regurgitation than those who received AS repair (20 (74.1%) vs. 7 (26.0%), P &lt; 0.001). AS repair was associated with a shorter duration of mechanical ventilation (20 vs. 29 hours, P = 0.039), faster discharge from the intensive care unit (2.0 vs. 4.0 days, P = 0.022) and shorter postoperative hospitalization (8.0 vs. 11.0 days, P = 0.008) compared with TAPE. Conclusions: APG demonstrated an upward trend in the TAPE group and a downtrend in the AS group after discharge from hospital. AS repair had an acceptable surgical effect in TOF patients, even in those with a dysplastic PVA. A higher APG remained upon hospital discharge in dysplastic patients with AS, but a downward trend was observed over time.

Pulmonary valve replacement via ministernotomy, early experience

The population of patients requiring pulmonary valve replacement (PVR) following surgery of the right ventricular outflow tract during infancy is growing. Full conventional sternotomy is widely used for PVR, while left anterior minithoracotomy has been described only in selected cases. Our aim is to describe our early experience with upper left ministernotomy as a systematic approach for PVR in teenagers and adult patients. From December 2019 to December 2021, 13 consecutive patients underwent PVR via ministernotomy at our institution. The indications were severe pulmonary regurgitation in 11 patients and severe conduit stenosis in 2 patients. Ten patients (76.9%) already had at least 1 sternotomy. Pulmonary homografts, stentless porcine bioprostheses or stented pericardial bioprostheses were implanted. Age at operation was 24.6 years old (17.6–38.4). There were no deaths, no cardiac injuries and no conversions to full sternotomy. The 3rd intercostal space was the most frequently used for the leftward extension of the ministernotomy ( Fig. 1 ). In 6 patients (46.1%), a residual intracardiac shunt was present and aortic cross-clamping was necessary. Median cardiopulmonary bypass and cross-clamping times were 70 (64–105.5) and 50 (40–75) minutes, respectively. Median intensive care and hospital stays were 2 (1–2.5) and 7 (6–11) days, respectively. All patients are doing well after a median follow-up of 16.2 months (13.5–19.0). Ministernotomy is a safe approach for PVR and redo PVR in teenagers and adults. This approach combines the advantages of minimally invasive surgery (expedited recovery) and those of a midline incision such as the access to the ascending aorta and the absence of additional scar in cases of redo PVR.

A RARE CASE OF DORV IN ADULTHOOD

Background: Double outlet right ventricle (DORV) is a rare cardiac malformation in children and even more rare when it comes to adulthood. Clinical Presentation: We report a boy with DORV with Mitral atresia and hypoplastic Left Ventricle who presented to us with breathlessness on climbing 2 flight of stairs at the age of 22 years.He was not seriously limited when he came to our hospital with complaint of breathlessness on exertion. The mother first noticed him getting breathless on climbing 2 flight of stairs at 5 years of age.The patient had history of recurrent infections in the past with failure to gain weight since childhood.On Examination the Pulse was 90 beats per minute,Spo2 was 85% on Room Air in all four limbs, BP was 90/60 mmhg, no tachypnea.There was Grade 2 clubbing present with cyanosis on the nails of all four limbs. Patient had cleft lip and cleft palate with pes cavus in left foot. On inspection, precordial bulge seen on left side with bony cage deformity and apex impulse in left 5th intercostal space lateral to midclavicular line and visible pulsations in 3rd intercostal space. On palpation, Diastolic thrill present in 2nd left intercostal space with grade 3 parasternal heave. On Auscultation, Grade 3/6 loud harsh diastolic murmur heard over the left precordium and interscapular and infrascapular area with diaphragm of stethoscope in supine position.Soft high pitched early diastolic murmur heard best at left upper sternal border and increased during inspiration. 2D echo suggestive of Double outlet Right ventricle with mitral atresia with hypoplastic left ventricle with pulmonary arterial hypertension and severe pulmonary regurgitation. Discussion: To our knowledge very few cases of DORV survives upto this age without any serious limitation that have been reported.DORV if diagnosed early and operated between the age of 1 year to 3 years have satisfactory results. Some cases that remain asymptomatic or are diagnosed late leads to development of pulmonary hypertension which makes them poor candidates of surgery.It was surprising as this patient have survived till now without any serious limitation or symptoms.

Left ventricular strain derived from cardiac magnetic resonance can predict outcomes of pulmonary valve replacement in patients with repaired tetralogy of Fallot.

PurposeSeveral adults with repaired tetralogy of Fallot (rToF) undergo pulmonary valve replacement (PVR) to reduce the right ventricular volume and retain right ventricular function. However, there is currently no consensus on the ideal time for PVR surgery in asymptomatic patients with rTOF with pulmonary regurgitation (PR). Clinical outcomes after PVR are also indeterminate. Recently, myocardial strain and strain rate derived from cardiac magnetic resonance (CMR) feature tracking were found to be more sensitive to right ventricular dysfunction than conventional parameters and therefore may add prognostic value in patients with rToF. We aimed to analyze whether pre-PVR left ventricular (LV) strain and strain rate detected by CMR feature tracking are associated with midterm outcomes after PVR in patients with rToF.MethodsSeventy-eight asymptomatic patients with rToF who required PVR due to moderate or severe PR were prospectively enrolled between January 2014 and June 2020. CMR cine sequences were obtained, and feature tracking parameters were measured preoperatively. Adverse events were documented during the follow-up. Receiver operating characteristic analysis was performed to determine the cutoff value. Kaplan–Meier curves were drawn with log-rank statistics; moreover, univariate and multivariate Cox proportional hazards regression analyses and Harrel C-indices were analyzed.ResultsDuring 3.6 ± 1.8 years of follow-up, 25 adverse events were recorded. Kaplan–Meier survival curves and univariate Cox analysis verified that patients with significantly reduced radial strain (RS), circumferential strain (CS), longitudinal strain (LS), RS rate at systole and diastole (RSRs and RSRe), and circumferential and LS rates at diastole (CSRe and LSRe) had worse event-free survival. After multivariate correction, only LS and LSRe remained significantly associated with adverse outcomes (hazard ratio = 1.243 [1.083–1.428] and 0.067 [0.017–0.258], respectively, all p < 0.05). The cutoff values of LS and LSRe were −12.30 (%) and 1.07 (s–1), respectively.ConclusionThe LV strain and strain rate prior to PVR are important prognostic factors for adverse events after PVR in rToF.

Outcomes of percutaneous balloon pulmonary valvuloplasty in pulmonary valve stenosis in the pediatric population in a single center, Lima - Peru

To evaluate the effectiveness of the procedure and outcomes during follow-up. 80 patients with pulmonary valvular stenosis who underwent percutaneous balloon valvuloplasty between January 2014 and December 2019 are described. Demographic, echocardiographic, and hemodynamic characteristics of the procedure were evaluated. Follow-up included clinical, echocardiographic parameters, pulmonary regurgitation severity, and residual pulmonary gradient at each cutoff point. The age range was 2 years (interquartile range: 10.5 months - 6 years), and the predominant sex was male with 56.2%. The transvalvular pulmonary gradient decreased from 61.7 mmHg +- 21.2 to 17 mmHg (interquartile range: 11-26 mmHg). The immediate success rate was 90%. Follow-up time showed a median of 21 months (interquartile range: 5-47.5 months). All patients at follow-up showed some degree of pulmonary insufficiency at each cutoff point; 17% of the cases at the end of their follow-up were found to have severe insufficiency. Three cases of long-term restenosis were found (3.8%), and 6 (7.5%) were admitted for valvuloplasty surgery or pulmonary valve replacement. The complications reported reached 10% of cases, two patients were admitted to surgery during the procedure for major complications. A significant association was found with severe pulmonary insufficiency at the end of follow-up and ring/balloon ratio. Percutaneous transluminal valvuloplasty with balloon is an effective technique in the treatment of pulmonary valvular stenosis, with reported complications but with good results during follow-up.

Hemodynamic effects of pulmonary regurgitation in one and one-half ventricle repair.

The pulmonary circulation after one and one-half ventricle repair is complex because of the direct connection of two sources of blood flow. Associated pulmonary regurgitation further complicates the pulmonary circulation. We report the complex hemodynamics depicted by phase-contrast magnetic resonance imaging in a patient with one and one-half ventricle circuit and severe pulmonary regurgitation. Antegrade superior vena caval flow occurred almost exclusively during diastole and regurgitated into the right ventricle. Consequently, the entire systemic venous return reached the right ventricle during diastole and is pumped back into the pulmonary arteries in systole. The case highlights the importance of analyzing flow patternsphase-by phase throughout the cardiac cycle instead of relying on the net flow volumes through each source of pulmonary blood flow.