Lutembacher Syndrome and Rheumatic Pulmonary Stenosis

We discribed a case of a 55-yr-old woman diagnosed with Lutembacher syndrome and rheumatic pulmonary stenosis. Congenital atrial septal defect was found in age 7 and rheumatic fever in age 34. As the patient developed pulmonary hypertension with calcified mitral valve leaflet and pulmonary stenosis so surgery was not indicated. So the patient was managed by medical therapy alone.

Balloon Angioplasty as a Modality to Treat Children with Pulmonary Stenosis Secondary to Complex Congenital Heart Diseases

Background： Pulmonary stenosis is common in children with complex congenital heart diseases. Proper management of this problem, especially postoperatively, is still controversial. This study was designed to assess the rate and determinants of success or failure of balloon angioplasty for such lesions. Methods： Clinical and hemodynamic data from 40 pediatric patients （24 boys and 16 girls） with complex congenital heart diseases who underwent balloon angioplasty were reviewed retrospectively from January 2012 to December 2016. Patients were divided into four groups according to the site of stenosis, which included pulmonary valve stenosis （PVS）, valved conduit stenosis, pulmonary artery stenosis （PAS）, and supravalvular pulmonary stenosis （SVPS）. Success rates were calculated according to defined criteria for initial success and favorable clinical impacts, and comparison between the successful subgroup and the unsuccessful subgroups was analyzed. Results： Grouped by the site of stenosis, initial success rates varied from 40.0% to 52.4% with the greatest success being seen in the PVS group, followed by the PAS group and SVPS group. In the PVS group and the PAS group, there was no statistical difference among age at dilation, postoperative interval, balloon/stenosis ratio, or pressure gradient predilation between the successful and the unsuccessful subgroups. Favorable clinical impacts included success rates of balloon angioplasty in the SVPS group, which was best （100%）, followed by the PVS group （90.9%） and the PAS group （85.7%）. There were a total of two transient complications （5.0%）. Conclusions： Balloon angioplasty was proven to be a safe and useful modality in children with complex congenital heart diseases and postoperative pulmonary stenosis, which should be the initial therapeutic modality in selected patients.

Pulmonary vein stenosis:Etiology,diagnosis and management

Pulmonary vein stenosis(PVS) is rare condition characterized by a challenging diagnosis and unfavorable prognosis at advance stages. At present, injury from radiofrequency ablation for atrial fibrillation has become the main cause of the disease. PVS is characterized by a progressive lumen size reduction of one or more pulmonary veins that, when hemodynamically significant, may raise lobar capillary pressure leading to signs and symptoms such as shortness of breath, cough, and hemoptysis. Image techniques(transesophageal echocardiography, computed tomography, magnetic resonance and perfusion imaging) are essential to reach a final diagnosis and decide an appropriate therapy. In this regard, series from referral centers have shown that surgical and transcatheter interventions may improve prognosis. The purpose of this article is to review the etiology, assessment and management of PVS.

Safety and efficacy of balloon angioplasty compared to stent-basedstrategies with pulmonary vein stenosis:A systematic review and meta-analysis

BACKGROUND Pulmonary vein stenosis(PVS)is an uncommon but known cause of morbidity and mortality in adults and children and can be managed with percutaneous revascularization strategies of pulmonary vein balloon angioplasty(PBA)or pulmonary vein stent implantation(PSI).AIM To study the safety and efficacy outcomes of PBA vs PSI in all patient categories with PVS.METHODS We performed a literature search of all studies comparing outcomes of patients evaluated by PBA vs PSI for PVS.We selected all published studies comparing PBA vs PSI for PVS with reported outcomes of restenosis and procedure-related complications in all patient categories.In adults,PVS following atrial fibrillation ablation and in children PVS related to congenital etiology or post-procedural PVS following total or partial anomalous pulmonary venous return repair were included.The patient-centered outcomes were risk of restenosis requiring re-intervention and procedural-related complications.The metaanalysis was performed by computing odds ratios(ORs)using the random effects model based on underlying statistical heterogeneity.RESULTS Eight observational studies treating 768 severe PVS in 487 patients met our inclusion criteria.The age range of patients was 6 months to 70 years and 67%were males.The primary outcome of the re-stenosis requiring re-intervention occurred in 196 of 325 veins in the PBA group and 111 of 443 veins in the PSI group.Compared to PSI,PBA was associated with a significantly increased risk of re-stenosis(OR 2.91,95%CI:1.15-7.37,P=0.025,I2=79.2%).Secondary outcomes of the procedurerelated complications occurred in 7 of 122 patients in the PBA group and 6 of 69 in the PSI group.There were no statistically significant differences in the safety outcomes between the two groups(OR:0.94,95%CI:0.23-3.76,P=0.929,I^(2)=0.0%).CONCLUSION Across all patient categories with PVS,PSI is associated with reduced risk of re-intervention and is as safe as PBA and should be considered first-line therapy for PVS.

Self-Expanding Nitinol Stent for Treatment of Tracheal Stenosis Caused by Pulmonary Hypertension:1 case report

A 38 year-old man was admitted because of half a year of recurrent bouts of eough and shortness of breath and 20 days of hemoptysis. He had been apparently healthy until the illness. In the recent 6 months, the dyspnea and fatigue gradually onset and became more severe, the hemoptysis being 100-200 mL per day. Spells of chest pain are associated with coughing. Despite accepting antibiotic and antispasmodic therapy in a hospital, there was no obvious improvement and he was transferred to our hospital. Physical examination on admission: He was in acute distress and anemic face With P 110/min., R 30/min., T36. 8oC and Bp 100/60 mmHg. There were Wheezes, medium and fine

Standardized Management of Acute Pulmonary Hemorrhage after Percutaneous Pulmonary Vein Intervention

Introduction:Pulmonary hemorrhage(PHm)is a life-threatening complication that can occur after catheter-based interventions in patients with pulmonary vein stenosis(PVS).Inhaled racemic epinephrine(iRE)and tra-nexamic acid(iTXA)have been used in other conditions,but a standardized approach in PVS has not been described.We aimed to describe the current management of PHm after PVS catheter-based interventions.Methods:We present a retrospective review of episodes of PHm from July 2022 to February 2024.PHm was defined as frank blood suctioned from the endotracheal tube including blood-tinged secretions and>3%decrease in saturations and/or ventilatory changes with or without acute chest X-ray changes.Each individual episode of PHm was considered a separate event.Incidence was calculated based on the total number of PVS interventions during the study period.Results:Eleven episodes of PHm were identified out of 108 PVS interventions,resulting in an incidence of 10.2%.Five(45.5%)had primary PVS,and seven(63.6%)had bilateral PVS.The median age at PHm was 23 months(3-91 months).Four episodes were treated with iRE,five with both iRE and iTXA,and two with only iTXA due to a history of suprasystemic right ventricular pressures.Median time on mechanical ventila-tion after PHm was 24 h(15-72 h)and a median ICU stay of 2 days(1-8 days).Hemostasis was achieved in all events.There were no adverse events after iTXA,however,transient hypertension was observed after iRE which was dose-related.Conclusions:The implementation of a standardized protocol for the treatment of PHm in PVS has the potential to improve procedural planning,has a wider availability of medications,and greater awareness by the providers involved,possibly leading to earlier detection of PHm and appropriate treatment.

Estimating pressure gradients by auscultation:How technology(echocardiography)can help improve clinical skills

AIM To extend our previously-published experience in estimating pressure gradients(PG) via physical examination in a large patient cohort. METHODS From January 1, 1997 through December 31, 2009, an attending pediatric cardiologist compared clinical examination(EXAM) with Doppler-echo(ECHO), in 1193 patients with pulmonic stenosis(PS, including tetralogy of Fallot), aortic stenosis(AS), and ventricular septal defect(VSD). EXAM PG estimates were based primarily on a murmur's pitch, grade, and length. ECHO peak instantaneous PG was derived from the modified Bernoulli equation. Patients were 0-38.4 years old(median 4.8).RESULTS For all patients, EXAM correlated highly with ECHO: ECHO = 0.99(EXAM) + 3.2 mm Hg; r = +0.89; P < 0.0001. Agreement was excellent(mean difference =-2.9 ± 16.1 mm Hg). In 78% of all patients, agreement between EXAM and ECHO was within 15 mm Hg and within 5 mm Hg in 45%. Clinical estimates of PS PG were more accurate than of AS and VSD. A palpable precordial thrill and increasing loudness of the murmur predicted highergradients(P < 0.0001). Weight did not influence accuracy. A learning curve was evident, such that the most recent quartile of patients showed ECHO = 1.01(EXAM) + 1.9, r = +0.92, P < 0.0001; during this time, the attending pediatric cardiologist had been > 10 years in practice.CONCLUSION Clinical examination can accurately estimate PG in PS, AS, or VSD. Continual correlation of clinical findings with echocardiography can lead to highly accurate diagnostic skills.

Immediate Therapeutic Outcomes and Medium-term Follow-up of Percutaneous Balloon Pulmonary Valvuloplasty in Infants with Pulmonary Valve Stenosis： A Single-center Retrospective Study

Background： Percutaneous balloon pulmonary valvuloplasty （PBPV） is the preferred therapy for pulmonary valve stenosis （PVS）. This study retrospectively reviewed recent PBPV outcomes in infants with PVS. The aim of this study was to evaluate factors associated with immediate therapeutic outcomes and restenosis during medium-term follow-up. Methods： The study included 158 infants with PVS who underwent PBPV from January 2009 to July 2015. Demographic characteristics and patient records were reviewed, including detailed hospitalization parameters, hemodynamic data before and immediately after balloon dilation, cineangiograms,and echocardiograms before PBPV and at each follow-up.All procedures were performed by more than two experienced operators. Results： Immediately after balloon dilation, the pressure gradient across the pulmonary valve decreased from 73.09 ± 21.89 mmHg （range： 43-151 mmHg） to 24.49 ± 17.00 mmHg （range： 3-92 mmHg; P 〈 0.001） and the right ventricular systolic pressure decreased from 95.34 ± 23.44 mmHg （range： 60-174 mmHg） to 52.07 ± 18.89 mmHg （range： 22-134 mmHg; P 〈 0.001）. Residual transvalvular pressure gradientsof67.31±15.19mmHg（range：50-92mmHg）werefoundin8.2%ofpatients,indicatingpoortherapeuticeffects;6.4%ofpatientshad variable-staged restenosis at follow-up and 3.8% underwent reintervention by balloon dilation or surgical repairs. Further analysis demonstrated that the balloon/annulus ratio showed statistically significant differences（P 〈 0.05） among groups with different therapeutic effects and between the restenosis and no-stenosis groups. Binary logistic regression analysis further revealed that higher balloon/annulus ratio （odds ratio： 0.005, 95% confidence interval： 0-0.39） was an independent protective factor for restenosis. The rate of severe complications was 1.9%. Conclusions： PBPV is a definitive therapy for infants with PVS based on its effectiveness, feasibility, and safety. Restenosis upon medium-term follow-up is relatively rare.

Transcatheter pulmonic valve implantation: Techniques, current roles, and future implications

Right ventricular outflow tract(RVOT)obstruction is present in a variety of congenital heart disease states including tetralogy of Fallot,pulmonary atresia/stenosis and other conotruncal abnormalities etc.After surgical repair,these patients develop RVOT residual abnormalities of pulmonic stenosis and/or insufficiency of their native outflow tract or right ventricle to pulmonary artery conduit.There are also sequelae of other surgeries like the Ross operation for aortic valve disease that lead to right ventricle to pulmonary artery conduit dysfunction.Surgical pulmonic valve replacement(SPVR)has been the mainstay for these patients and is considered standard of care.Transcatheter pulmonic valve implantation(TPVI)was first reported in 2000 and has made strides as a comparable alternative to SPVR,being approved in the United States in 2010.We provide a comprehensive review in this space–indications for TPVI,detailed procedural facets and up-to-date review of the literature regarding outcomes of TPVI.TPVI has been shown to have favorable medium-term outcomes free of reinterventions especially after the adoption of the practice of pre-stenting the RVOT.Procedural mortality and complications are uncommon.With more experience,recognition of risk of dreaded outcomes like coronary compression has improved.Also,conduit rupture is increasingly being managed with transcatheter tools.Questions over endocarditis risk still prevail in the TPVI population.Head-to-head comparisons to SPVR are still limited but available data suggests equivalence.We also discuss newer valve technologies that have limited data currently and may have more applicability for treatment of native dysfunctional RVOT substrates.

Important Newborn Cardiac Diagnostic Dilemmas for the Neonatologist and Cardiologist-A Clinical Perspective

Most congenital heart disease(CHD)is readily recognisable in the newborn.Forewarned by previous fetal scanning,the presence of a murmur,tachypnoea,cyanosis and/or differential pulses and saturations all point to a cardiac abnormality.Yet serious heart disease may be missed on a fetal scan.There may be no murmur or clinical cyanosis,and tachypneoa may be attributed to non-cardiac causes.Tachypnoea on day 1 is usually non-cardiac except arising from ventricular failure or a large systemic arteriovenous fistula.A patent ductus arteriosus(PDA)may support either pulmonary or systemic duct dependent circulations.The initially high pulmonary vascular resistance(PVR)limits shunts so that murmurs even from large communications between the systemic and pulmonary circulations take days/weeks to develop.At times despite expert input,serious CHD maybe difficult to diagnose and warrants close interaction between the neonatologist and cardiologist to reach a timely diagnosis.Such conditions include obstructed total anomalous pulmonary venous connections(TAPVC)and the need to distinguish it from persistent pulmonary hypertension in the newborn(PPHN)–the treatment of the former is surgical the latter medical.A large duct shunting right to left may overshadow a suspected hypoplastic aortic isthmus and/or coarctation.Is the right to left shunting because of severe aortic obstruction or resulting from a high PVR with little obstruction.The diagnosis of pulmonary vein stenosis(PVS)remains problematic often developing in premature infants with ongoing bronchopulmonary dysplasia(BPD),still being cared for by the neonatologist.While there are other diagnostic dilemmas including deciding the contribution of a recognised CHD in a sick neonate,this paper will focus on the above-mentioned conditions with suggestions on what may be done to arrive at a timely diagnosis to achieve optimal outcomes.

Unilateral pulmonary artery stenosis and late-onset cataract in an adult： a case of suspected congenital rubella syndrome

Congenital rubella syndrome is characterized by the triad of deafness, cataract and cardiovascular mafformations. The great majority of the cases in the literature have been usually diagnosed in infancy and childhood because of various defects at birth. However, we report a rare case of suspected congenital rubella syndrome in an adult with unilateral pulmonary artery stenosis and late-onset cataract.

Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery:A case report

BACKGROUND Cornelia de Lange syndrome(CdLS)is a congenital multisystemic genetic disorder.The expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent years.However,they still more frequently undergo cardiac surgery.There are some challenges for clinicians when faced with CdLS patients.We present the perioperative management of a child with CdLS undergoing open-heart surgery.CASE SUMMARY Severe pulmonic and subpulmonic valvular stenosis,enlargement of the right side of the heart,mild tricuspid regurgitation,atrial septal defect,and patent ductus arteriosus were diagnosed in a 14-month-old boy with manifested cyanosis,developmental delay,and malnutrition.Attempted balloon valvuloplasty was unsuccessful due to a severe stenotic pulmonary valve,therefore it was decided to perform an open surgical repair.Following a successful and uncomplicated intraoperative course,the patient was extubated on postoperative day 5,and adrenalin and dopamine infusions were gradually decreased and stopped on postoperative days 6 and 10,respectively.Moderate laryngomalacia and suboptimal vocal cord movements were diagnosed,and tracheotomy and percutaneous endoscopic gastrostomy were performed under general anesthesia in the same session at postoperative day 32.The patient was discharged on postoperative day 85 after a challenging postoperative period with additional airway and nutritional problems.CONCLUSION This is the first report of the perioperative anesthetic and clinical management of a CdLS patient undergoing open-heart surgery.