A successful percutaneous valvuloplasty in an 80-year-old woman with severe pulmonary stenosis.

Echocardiographic Determinants of Clinical Course in Infants With Critical and Severe Pulmonary Valve Stenosis

Objective To evaluate the effectiveness of urgent surgical procedures for severe pulmonary valve stenosis in infants and children. Methods Seventy-four (51 male, 23 female) children with severe pulmonary valve stenosis who received surgical correction were collected from Oct.2006 to Jan.2013.The age of patients ranged from 2 months to 13 years(median age was 26 months), and the body weight was 5-47 kg (median weight was 16 kg). Patients had other cardiac deformities, and among them 20 patients were associated with the patent ductus arteriosus(PDA), 22 cases with patent foramen ovale (PFO), 32 cases with atrial septal defect (ASD), 13 cases with ventricular septal defect (VSD), and 4 cases with double-chambered right ventricle.Radical corrections were performed in all patients under hypothermic general anaesthesia with cardiopulmonary bypass.There were 46 patients undergoing transannular patching with autologous pericardial flake and 28 patients undergoing transarterial pulmonary valvotomy. Results Urgent operations were performed in all patients and no death occurred during surgery.Median bypass time was 72 minutes(ranging from 40 to 100 minutes) and median aortic cross-clamp time was 43 minutes(ranging from 13 to 79 minutes). The time for the use of respirator was 4-128 h (median time was 15 h), and ICU stay lasted 4-18 days(median time was 5 days). Perioperative complications: included 1 case of ventricular fibrillation during operation, after electrical shock the patient returned to normal; postoperative complications included low cardiac output syndrome(4 cases), refractory hypoxemia(8 cases), acute renal failure(1 case), pneumothorax(2 cases), hydrothorax(2 cases), atelectasis(1 case). All patients received chest radiography, electrocardiogram and echocardiography during hospital stay.As a result, postoperative cardiac function was improved significantly. Conclusions Low cardiac output syndrome and hypoxemia aften exist in severe pulmonary stenosis.If severe pulmonary stenosis is diagnosed and operated early, the prognosis is good.The key to this kind of surgery is the application of autologous pericardium or disc pericardium patching for widening pulmonary artery appropriately, which fully rescind the right ventricular outflow tract obstruction.At the same time, the level of postoperative monitoring should be improved, and postoperative complications must be handled timely. Key words: Severe pulmonary valve stenosis; Cardiac surgical treatment; Congenital heart disease

Continuous Spinal Analgesia with Sufentanil for Labor and Delivery in a Parturient with Severe Pulmonary Stenosis

Rationale: Noonan syndrome is a genetic disorder with variable clinical manifestations that affects different systems. Among the multitude of clinical findings, the main characteristics are unique facial traits, low height, and congenital cardiac abnormalities. Recognizing such a syndrome is of great importance because of its involvement of multiple organs. Therefore, a systematic and thorough evaluation according to the guidelines for the assessment and management of Noonan syndrome is needed. Patient concerns: A female infant, 3 months old, presented with a difficult neonatal period (respiratory distress, generalized cyanosis), typical facial dysmorphia, evolutionary height hypotrophy, persistent leukocytosis, factor VII deficit, and severe pulmonary stenosis. Diagnosis: Echocardiography showed severe pulmonary valve stenosis, mild pulmonary regurgitation, a 6.5 mm ostium secundum atrial septal defect, and a persistent ductus arteriosus. Laboratory reports revealed leukocytosis with monocytosis, anemia, and thrombocytopenia, but no infectious source was found. Interventions: In this context, a pulmonary balloon valvuloplasty was performed, with a significant decrease of the transpulmonary gradient. Outcome: The patient was genetically tested, and the reports were positive for Noonan syndrome (mutation in exon 13 of PTPN 11). At 3years of age, she underwent open-heart repair of the atrial septal defect and significant recurrent pulmonary stenosis. The postoperative course was uneventful, and the patient remained asymptomatic at 6months follow-up. Conclusions: The diagnosis was initially suggested by the characteristic unique clinical events associated with cardiac involvement and confirmed later through genetic molecular testing. Prompt treatment of the severe life-threatening pulmonary valve stenosis was important, and the patient underwent additional interventions, after which she remained stable.

Historically, right-sided valvular disease has received less attention from clinicians and researchers than left-sided valve disease, in part because of a protracted latent asymptomatic period. Moreover, because tricuspid regurgitation (TR) is often due to left-sided valve disease and pulmonary regurgitation (PR) is often secondary to congenital cardiac disease, the underlying disorder rather than the valve lesion tends to dominate the clinical picture. It is increasingly recognized that right-sided valve disease is not benign and has a significant and independent impact on morbidity and mortality. Today, diagnostic techniques and appropriate management strategies for patients with right-sided valve disease are established and continually refined. In this era of increasing awareness and improved treatment options for patients with valve disease, it is important that clinicians consider the diagnosis of right-sided valve disease, understand its pathophysiology, choose appropriate confirmatory testing, and refer patients for timely intervention to prevent clinical deterioration with associated adverse consequences. This review examines the causes of right-sided valve disease and the latest diagnostic advances and treatment options for these often-neglected valve lesions. Patients with native right-sided valve disease are rarely affected by endocarditis, and indications for prophylaxis have recently changed.1 ### Tricuspid Regurgitation TR that is at least moderate in severity is most frequently “functional” in nature and by definition not related to primary tricuspid valve (TV) leaflet pathology but rather secondary to another disease process causing right ventricular (RV) dilatation, distortion of the subvalvular apparatus, tricuspid annular dilatation, or a combination of these. Furthermore, a moderate or greater degree of TR, regardless of primary origin, usually engenders additional TR as a result of the adverse hemodynamic consequence of RV volume overload. Causes of clinically significant TR are outlined in Table 1; 2 classic examples of primary tricuspid leaflet pathology are demonstrated in Figure 1. View this table: Table 1. Causes of TV …

A nine-year-old, domestic shorthair cat was referred for investigation of a suspected renal mass, polyuria, polydipsia, hyporexia and weight loss of one month's duration; no respiratory signs were reported. On presentation, the cat had marked respiratory effort. Thoracic auscultation revealed reduced heart and lung sounds bilaterally. Transthoracic echocardiography revealed a large pleural effusion and an extensive, homogenous mass within the right ventricular outflow tract, invading the pulmonary valve and causing severe infundibular and valvular pulmonary stenosis and right atrial and ventricular dilatation. The mass extended to, and infiltrated, the right ventricular free wall. Postmortem examination confirmed the presence of a mass associated with the pulmonary valve extending into the right ventricle and infiltrating the right ventricular and right atrial myocardium, resulting in severe infundibular and valvular pulmonary stenosis. Histopathology showed disseminated intermediate to large cell lymphoma affecting the kidney, myocardium, pulmonary valve, pancreas, diaphragm and adrenal glands.

We present a 31 gestational weeks' premature baby whose fetal echocardiogram showed ectopia cordis, single ventricle and severe pulmonary stenosis. At 31 gestational weeks, an emergency caesarean section was performed, and his birth weight was 1756 g, SpO2 was 80% on 100% O2. Epicardial echocardiogram showed double inlet right ventricle, severe valvular pulmonary stenosis and no ductus arteriosus. The risk of surgery was very high, so we decided to perform balloon valvuloplasty by direct puncture of the heart. We punctured the apex of the ventricle using a 16-gauge needle under echo guidance, advanced the guidewire to the pulmonary artery and performed balloon valvuloplasty. Soon after the procedure, the cyanosis improved dramatically. This is the first report of a transcatheter procedure performed by direct puncture of the heart for ectopia cordis with complex congenital heart disease.

Objective To study the clinical features and risk factors of acute pulmonary reperfusion injury after operation in neonates with severe pulmonary stenosis or pulmonary atresia. Method From February 2014 to February 2018, a retrospective analysis was performed in patients with critical pulmonary stenosis or pulmonary atresia who received percutaneous balloon pulmonary valvuloplasty (PBPV) in the neonatal intensive care unit of our hospital. Clinical characteristics, perioperative cardiac structure, hemodynamic data and biochemical results were collected. The neonates were assigned into injury group if they had acute lung reperfusion injury, and non-injury group if not. The risk factors of acute lung reperfusion injury were analyzed using multi-variate Logistic regression model. Result A total of 32 patients (24 prenatal diagnosis and 8 postnatal diagnosis) with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum were enrolled. The main manifestations were dyspnea and cyanosis. Intravenous prostaglandin E was administered to keep the ductus arteriosus open. The age of operation ranged from 1 to 52 days and the median age was 7.5 days. Postoperative acute lung reperfusion injury occurred in 7 cases (21.9%). Preoperative and intraoperative pulmonary valve annulus diameter, balloon diameter, preoperative hemoglobin, hematocrit and blood albumin were significantly lower in the injury group. The operation duration, total length of hospital stay and postoperative duration were longer than in the non-injury group, the differences were statistically significant (P<0.05). Multi-variate Logistic regression analysis showed that the diameter of pulmonary valve annulus (OR=5.814, 95%CI 1.106~30.568), preoperative blood albumin (OR=1.361, 95%CI 1.063~1.742), and hematocrit (OR=1.173, 95%CI 1.010~1.363) were risk factors of acute lung reperfusion injury, with statistically significant differences (P<0.05). Conclusion Acute lung reperfusion injury is one of the common complications after the operation of severe pulmonary stenosis or pulmonary atresia. The severity of pulmonary valve annulus stenosis, preoperative hematocrit and blood albumin level may be the risk factors of postoperative acute lung reperfusion injury. Key words: Reperfusion injury; Pulmonary valve stenosis; Pulmonary atrasia; Percutaneous pulmonary balloon valvuloplasty

Severe pulmonary stenosis or atresia with intact ventricular septum falls within the group of cardiac malformations which are characterized functionally by obstruction to pulmonary flow at the right side of the heart. This malformation is commonly manifested clinically by intense cyanosis, cardiac failure, and death in the neonatal period. Whereas the diagnosis and management of pulmonary stenosis in older children and adults is well established, there are relatively few published data relating to atresia or severe stenosis in infants having neonatal difficulties. In 1951, Johnson and Johnson 1 reported 5 patients with pulmonary stenosis who died between 3 and 19 months of age. Subsequently, others 2-4 having similar experiences have stressed the importance of early diagnosis and surgical correction. Pulmonary atresia (complete fusion of valve cusps) and severe pulmonary stenosis (extremely tiny or pinpoint orifice) may present similar or identical pictures. Cases with pulmonary atresia or severe stenosis may be

The natural history of pulmonary valvular stenosis: Serial cardiac catheterizations in 21 children

Rationale:Patients of critical pulmonary artery stenosis would face severe hypoxemia, cardiac failure as well as massive hemorrhage during percutaneous balloon dilation and pulmonary arterial stent implantation. Here, we present a case in which the elective use of extracorporeal membrane oxygenation (ECMO) support successfully facilitated safe percutaneous balloon dilation of pulmonary artery and stent implantation on a patient with severe pulmonary artery stenosis caused by aorto-arteritis.Patient concerns:A 47-year-old man was hospitalized due to 10 years of post-exercise exhaustion and shortness of breath. Half a month ago the symptoms deteriorated. He also manifested systemic edema and could only sit upright to breath during night time. Computed tomographic angiography (CTA) indicated severe pulmonary stenosis caused by aorto-arteritis.Diagnoses:Right pulmonary artery stenosis, left pulmonary artery occlusion, severe tricuspid regurgitation, right atrium, and ventricle enlargement, atrial fibrillation with rapid ventricular rates, NYHA class IV, pulmonary infection.Interventions:V-A ECMO support was considered during percutaneous balloon dilation of pulmonary artery and stent implantation.Outcomes:The patient remained hemodynamically stable throughout the procedure with no inotropic support. ECMO was successfully weaned off after the intervention, with no procedural complications. Postoperative echocardiography indicated much better heart function, and he was discharged uneventfully 5 days later.Conclusion:V-A ECMO is capable of preventing hypoxemia and providing effective circulation support during cardiac intervention in patients of severe pulmonary stenosis.

Twenty-six patients in infancy and early childhood with severe pulmonary valve stenosis and intact ventricular septum are reviewed. They were selected from a larger series of 112 patients with pulmonary stenosis of any degree, on account of early onset of symptoms and the severity of the stenosis proven by cardiac catheterization and angiocardiography, at operation or at necropsy. Our criteria for severity in this series were: presence of symptoms within the first two years of life; right ventricular and right atrial hypertrophy on electrocardiography; and right ventricular pressure equal to or higher than systemic blood pressure. The warning signs prompting valvotomy are deterioration of the following features: cyanosis and dyspnoea; congestive cardiac failure; tricuspid incompetence; cardiac enlargement and pulmonary oligaemia on radiograph; and right ventricular and right atrial hypertrophy on electrocardiography. The lives of 13 patients were saved by timely valvotomy. These patients are all well six months to six years after operation. Five patients died before any operation could be performed. Eight patients died within 48 hours of operation. Had some of these patients been operated on earlier the evidence indicates that they would have had a better prognosis. Therefore the importance of early recognition, prompt treatment, and emergency valvotomy, if necessary, is emphasized.

Patient: Male, 40-year-oldFinal Diagnosis: ccTGASymptoms: DyspneaMedication: —Clinical Procedure: —Specialty: CardiologyObjective:Congenital defects/diseasesBackground:Congenitally corrected transposition of great arteries (ccTGA) represents a distinct rare group of congenital heart diseases. Survival of unoperated ccTGA in the presence of large ventricular septal defect (VSD) is exceptional. Furthermore, late presentation of such patients in the absence of severe pulmonary hypertension or severe systemic right ventricle dysfunction is unusual.Case Report:We report a rare late presentation of ccTGA associated with large VSD in the absence of severe pulmonary hypertension or systemic ventricle dysfunction. An associated severe pulmonary valve stenosis maintained a balanced and stable condition up to the fourth decade of life. The patient has also dextrocardia, which is an unusual association. The diagnosis was reached using multimodality imaging including transthoracic echocardiogram (TTE), transesophageal echography (TEE), cardiac magnetic resonance imaging (CMR), and cardiac computed tomography (cardiac CT).Conclusions:The presence of pulmonary stenosis can provide a physiological protection that avoids unnecessary surgical correction of large VSD in ccTGA patients. However, such a decision should be made on an individual basis and following a careful anatomical and functional evaluation.

Objectives : this study aim to assess the effectiveness of the Transcatheter balloon valvoplasty (BVP) of severe Pulmonary stenosis (PS) and identify possible complications and predictors for the outcome. Methods : A retrospective study was conducted at the Iraqi Center of Cardiac Diseases for a 2-year duration in the period from 2021 to the end of 2023; the patients with confirmed severe PS of both genders within the age equal to or less than 18 years were included. For every patient included in the present study, the following data were collected: Demographic data, Echocardiographic criteria Hemodynamic parameters pre- and post-Cath, Catheterization data including the site of vascular access, need for general anesthesia and tracheal intubation, number and size of balloons used, any complications recorded at therapeutic session obtained from Cath lab archive. Results : After BVP, the pressure gradient across the valve reduced from 105±25 to 32 ± 11(mmHg), (P&lt;0.001); the systemic right ventricular pressure dropped from 120 ± 25 (mmHg) before to 54 ± 14 (mmHg) after (P&lt;0.001). A significant increase in the systemic pulmonary pressure from 18 ± 4 (mmHg) before to 22 ± 3(mmHg) (P&lt;0.001). Tricuspid valve regurgitation (TVR) reduced significantly (P=0.002). Pulmonary incompetence (PI) changed from mild to moderate and severe in 12 cases (P=0.001). Nine cases (20%) developed complications; 6 (13.3%) supraventricular tachycardia and 3 (6.7%) Cardiac arrest and cardiopulmonary resuscitation. The strongest association to complication occur-rence was pulmonary valve annulus size with r= 0.417 and TVR and PI severity with P=0.001, 0.017, respectively. Conclusion : Transcatheter BVP is an effective procedure for severe PS cases but carry a risk of cardiac complications. The complication rate was linked to the patients’ demographical characteristics and PV annulus size and degree of TVR and PI severity.

Congenital pulmonary valve stenosis is a common congenital heart disease andIsolated pulmonary valve stenosis comprises 8-10% of all congenital heart disease. It is an acyanotic heart disease,but can present with severe cyanosis if it is associated with patent foramen ovale (PFO). Severe pulmonary stenosis with cyanosis can be misdiagnosed clinically. Proper evaluation and modern technique of treatment modality can save a life easily. We report a 5 years old boy with severe pulmonary valvular stenosis with PFO who was clinically misdiagnosed as a case of congenital cyanotic heart disease (Tetralogy of Fallot). After taking proper history, clinical examination and investigations we treated the baby by balloon pulmonary valvuloplasty (BPV) successfully without any complication. The short term (6 months) outcome of BPV showed excellent result.&#x0D; Ibrahim Cardiac Med J 2013; 3(1&amp;2): 53-58