Infundibular Pulmonary Stenosis Research Articles

Pulmonary Infundibular Stenosis Secondary to a Mediastinal Hodgkin Lymphoma Tumor Invasion in a Young Adult Male: What a Rare Occurrence!

Introduction: Cardiac involvement is a rare unusual localization of Hodgkin lymphoma, as it’s known that primary lymphomas of the heart are rare. They develop primarily in the right atrium, and extend to the pericardium in about one third of the cases. Pulmonary infundibular stenosis secondary to mediastinal mass Hodgkin lymphoma invasion is rare but not impossible especially in relapse cases. Case Report: A rare case of a young adult patient diagnosed with Hodgkin Lymphoma under chemotherapy presents a relapse of his pathology associated with heart failure; predominantly, right heart failure due to extension of the mediastinal mass tumor to the right cavities of the heart causing pulmonary infundibular stenosis which is a rare occurrence. He was put under an intense chemotherapy and symptomatic heart failure treatment with close follow-up after multi-disciplinary discussions including cardiologist, onco-hematologists and cardiothoracic surgeons. Three months later, a significant reduction of the mass was observered. Conclusion : Pulmonary infundibular stenosis secondary to mediastinal Hodgkin lymphoma invasion is a rare entity of secondary tumors of the heart. Multimodal imagery is the key tool for diagnosis. There prognosis depend on multiple factors but generally good in younger adult patients compared to aged individuals.

Clinical Presentation and Therapy of Tetralogy of Fallot and Double-Outlet Right Ventricle.

Tetralogy of Fallot (TOF) is the most common cyanotic heart defect. TOF consists of the combination of four anomalies (Fig. 35.1): (1) a large malalignment ventricular septal defect, (2) an obstruction of the right ventricular outflow tract (usually infundibular and valvular pulmonary stenosis with a small pulmonary valve annulus and supravalvular stenosis, (3) an aorta that "overrides" the ventricular septal defect, and (4) right ventricular hypertrophy. TOF represents 4-8% of congenital heart defects. Specific variations of TOF include all forms of pulmonary atresia with VSD and absent pulmonary valve syndrome. In addition, the left and right main pulmonary arteries may be stenotic or hypoplastic. In these cases, there may be major aortopulmonary collateral arteries (MAPCAs) which are vessels arising from the aorta or the subclavian arteries that supply segments of the pulmonary arterial tree. Additional variations include an ASD (Pentalogy of Fallot), a right aortic arch, and coronary abnormalities.

Congenital absence of sternum with tetralogy of Fallot, right aortic arch, and bilateral superior vena cava

BackgroundCongenital complete absence of sternal bone or cleft sternum or absent sternum is a very rare and potentially life-threatening birth defect. An association of cleft sternum with tetralogy of Fallot, right aortic arch, and bilateral superior vena cava is extremely rare, and not many cases have been reported.Case presentationA 2-year-old child was brought for routine checkup. He was a known case of congenital heart disease and midline thoracic defect at age of 3 months. Further investigations revealed absent sternum with tetralogy of Fallot, large cono-ventricular defect with bidirectional shunt, severe infundibular pulmonary stenosis, superiorly located right aortic arch, and bilateral superior vena cava.ConclusionCongenital absence of the sternum with intracardiac defects like tetralogy of Fallot with right aortic arch and bilateral superior vena cava is extremely rare malformation. Early diagnosis and surgical correction give the infant the best chance of survival.

Cardiovascular Abnormalities Associated with Heterotaxy Syndrome: A Case Series

Abstract Heterotaxy syndromes result from the failure of normal lateralization, resulting in the abnormal arrangement of cardiothoracic and abdominal viscera. These are associated with a disproportionate morbidity and mortality rate, mandating early diagnosis, and surgical intervention. Accurate determination of situs, as well as congenital abnormalities, is necessary for surgical planning and determination of postoperative outcome and quality of life. In this case series, we present the spectrum of cardiac, pulmonary, and systemic vascular abnormalities associated with heterotaxy syndromes. Cardiac computed tomography angiography (CTA) was performed on 10 patients with heterotaxy syndromes diagnosed on echocardiography. We found that the most common congenital heart disease associated with heterotaxy in our case series was atrioventricular septal defect. Among the extracardiac vascular abnormalities, pulmonary infundibular stenosis, right-sided aortic arch, and double superior vena cava were common. Moreover, not all cases displayed congruence between cardiac, tracheobronchial, and visceral situs. In conclusion, CTA is useful in the diagnosis of intracardiac and extracardiac vascular abnormalities associated with heterotaxy syndromes. In addition, it gives valuable information about the tracheobronchial situs, visceral situs, and other congenital abnormalities.

Infective Endocarditis Caused by Granulicatella elegans in a Child With Patent Ductus Arteriosus.

To the editors: Infective endocarditis (IE) is an inflammatory process of infectious origin that affects cardiovascular structures. The term endarteritis is currently included in the definition of infective endocarditis. Granulicatella elegans is a rare cause of infective endocarditis, which has a subacute presentation of the disease, with nonspecific symptoms and mild elevation of inflammatory biomarkers. It is associated with a high-mortality rate in adults.1 In the pediatric age group, there are 3 cases of IE described by Granulicatella spp., associated with congenital heart disease: 1 with pulmonary atresia with intact interventricular septum and mild Ebstein’s malformation already operated,2 another one with Shone’s syndrome and the last one with infundibular pulmonary stenosis.1 Our case represents the fourth case worldwide with IE caused by Granulicatella spp., in congenital heart disease, and the first with patent ductus arteriosus (PDA). We report an 8-year-old female patient with a history of PDA was diagnosed at another hospital at 3 years of age. She was referred from another hospital with a 7-month history of fever, predominantly during the evening, between 38.4°C and 38.6°C. On admission hyperdynamic precordium with thrill, normal S2, left infraclavicular continuous murmur grade 4/6, slightly grainy sound, calling attention to a metallized acoustic component and wide pulses. Three blood cultures tested positive after 48 hours. Gram staining of the blood culture revealed Gram-positive cocci, that were, nonsporulating in pairs and short chains and, pleomorphic (Fig. 1A). Blood cultures were identified with Granulicatella elegans nutritionally variant streptococci using a commercial kit MALDI Sepsityper (Bruker Daltonics). Little growth on sheep-blood agar in a microaerophilic atmosphere after 72 hours.FIGURE 1.: A. Gram staining showing Gram-positive cocci, nonsporulating in pairs and, short chains, pleomorphic. B, (day 30) TTE. Parasternal 2D short axis view and color Doppler showing PDA with left to right shunt and hyperechogenic mass (green arrow) in the PA following the flow of the ductus. PA, pulmonary artery; PDA, patent ductus arteriosus; TTE, transthoracic echocardiogram.Vegetations were not found on the transthoracic echocardiogram (TTE) at admission, but after 30 days reveled; image suggestive of vegetation attached the pulmonary trunk (13 mm × 3 mm) (Fig. 1B). After completing 40 days of ceftriaxone, the vegetation was removed percutaneously, on the 58th day, the PDA was closed with Amplatzer ADO 8/6. Despite advances in diagnosis and treatment, infective endocarditis in the 21st century can be a life-threatening disease with high morbidity and mortality.1 Cyanogenic congenital heart disease, left heart lesions and atrioventricular defects are associated with an increased risk of IE in childhood.3 In children, the most frequent microorganism in subacute IE is Streptococcus viridans group4; however, in the second group nutritionally variant Streptococcus species (NVS) have been identified as etiological agents responsible for 5% of the cases with bacteriologic diagnosis.5 Physicians should suspect subacute IE in patients presenting with intermittent fever of prolonged evolution, constitutional symptoms, and changes in auscultatory phenomena in the context of children with congenital heart disease, despite having negative initial blood culture and absence of initial IE findings on echocardiogram. Granulicatella spp., IE has a subacute presentation with a mild increase in inflammatory biomarkers, and its morbidity and mortality can be significant. The modified Duke criteria are of great value; however, in some patients with subacute IE the diagnosis can be missed, therefore, the clinical features should be considered when a patient has a fever of unknown origin.

‘Ring’ in the Left Ventricular Outflow Tract

A 4-year-old boy (weight 17.1 kg, height 107 cm) presented with feeding difficulty, shortness of breath, and cyanosis. The preoperative transthoracic echocardiography examination showed a small perimembranous ventricular septal defect, severe right ventricular infundibular pulmonary stenosis with a peak gradient of 140 mmHg, right ventricular hypertrophy, right-to-left shunt, and good biventricular function. The pulmonary valve, main pulmonary artery, and branch pulmonary arteries were normal. The child was scheduled for right ventricular outflow tract infundibular resection and ventricular septal defect closure.

RAF-1 Mutation Associated with a Risk for Ventricular Arrhythmias in a Child with Noonan Syndrome and Cardiovascular Pathology.

IntroductionNoonan syndrome (NS) is a dominant autosomal disease, caused by mutations in genes involved in cell differentiation, growth and senescence, one of them being RAF1 mutation. Congenital heart disease may influence the prognosis of the disease.Case presentationWe report a case of an 18 month-old female patient who presented to our institute at the age of 2 months when she was diagnosed with obstructive hypertrophic cardiomyopathy, pulmonary infundibular and pulmonary valve stenosis, a small atrial septal defect and extrasystolic arrhythmia. She was born from healthy parents, a non-consanguineous marriage. Due to suggestive phenotype for NS molecular genetic testing for RASopathies was performed in a center abroad, establishing the presence of RAF-1 mutation. Following rapid progression of cardiac abnormalities, the surgical correction was performed at 14 months of age. In the early postoperative period, the patient developed episodes of sustained ventricular tachycardia with hemodynamic instability, for which associated treatment was instituted with successful conversion to sinus rhythm. At 3-month follow-up, the patient was hemodynamically stable in sinus rhythm.ConclusionsThe presented case report certifies the importance of recognizing the genetic mutation in patients with NS, which allows predicting the severity of cardiac abnormalities and therefore establishing a proper therapeutic management of these patients.

Neurofibromatosis 1-Noonan Syndrome Associated with Pulmonary Stenosis and Hypertrophic Cardiomyopathy

Abstract Neurofibromatosis 1-Noonan syndrome is considered a distinct clinical entity, combining characteristics of both autosomal dominant disorders: neurofibromatosis 1 and Noonan syndrome. We present the case of a 20-year-old patient clinically diagnosed with neurofibromatosis 1-Noonan syndrome, with genetic confirmation-heterozygous mutation of PTPN11 gene and a variant of uncertain significance in NF1 gene (c.2989A>G). Associated congenital heart disease was diagnosed at birth-severe pulmonary valve stenosis and infundibular pulmonary stenosis, surgically corrected at the age of one. At adult age, cardiologic assessment shows severe pulmonary regurgitation post commissurotomy, with residual large pulmonary stenosis and left ventricular apical hypertrophy, suggesting apical hypertrophic cardiomyopathy, confirmed by cardiac magnetic resonance. The patient needs periodical follow-up in order to identify the perfect timing for correction of severe pulmonary regurgitation. As there are no specific genetic therapies for neurofibromatosis 1 or Noonan syndrome, the diagnose and management of associated comorbidities is the main aspect to be taken into considered.

C11. Aorto-Pulmonary Window in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome : A Rare Case Report

Abstract Background Aorto-pulmonary window and Tetralogy of Fallot with absent pulmonary valve syndrome (TOF-APVS) are very rare entities. The combination of the two defects in adolescence patient made the diagnosis based on standard imaging modalities challenging. Case Illustration 14-year-old boy was referred from Borneo Hospital with failure to thrive and shortness of breath symptoms. He was underwent multiple modalities procedure and it shows the presences of features of tetralogy of Fallot : right ventricular hypertrophy, sub-aortic ventricular septal defect and pulmonary infundibular stenosis. Pulmonary valve leaflets are not seen with mild dilatation of the main and left pulmonary artery (Absent PV syndrome) and a connection from Pulmonary Artery and Ascending Aorta (Aorta-Pulmonary Window) Discussion TOF-APVS is a relatively rare variant of TOF. Its co-existence with Aorto-Pulmonary Window make this case was very exceptional. There are only a few reported cases diagnosed with TOF-APVS in adult age in the literature as most of them are diagnosed at younger ages. The unique physiology due to the large connection of Ascending aorta and the main pulmonary artery resulting in L-R Shunt. The large connection from Ascending Aorta to Main Pulmonary artery (L-R Shunt) prompt this patient to pulmonary hypertension (before and after surgery) and heart failure, hence initial treatment of ACE-I and sildenafil was very necessary.

C11. Aorto-Pulmonary Window in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome : A Rare Case Report

Abstract Background Aorto-pulmonary window and Tetralogy of Fallot with absent pulmonary valve syndrome (TOF-APVS) are very rare entities. The combination of the two defects in adolescence patient made the diagnosis based on standard imaging modalities challenging. Case Illustration 14-year-old boy was referred from Borneo Hospital with failure to thrive and shortness of breath symptoms. He was underwent multiple modalities procedure and it shows the presences of features of tetralogy of Fallot : right ventricular hypertrophy, sub-aortic ventricular septal defect and pulmonary infundibular stenosis. Pulmonary valve leaflets are not seen with mild dilatation of the main and left pulmonary artery (Absent PV syndrome) and a connection from Pulmonary Artery and Ascending Aorta (Aorta-Pulmonary Window) Discussion TOF-APVS is a relatively rare variant of TOF. Its co-existence with Aorto-Pulmonary Window make this case was very exceptional. There are only a few reported cases diagnosed with TOF-APVS in adult age in the literature as most of them are diagnosed at younger ages. The unique physiology due to the large connection of Ascending aorta and the main pulmonary artery resulting in L-R Shunt. The large connection from Ascending Aorta to Main Pulmonary artery (L-R Shunt) prompt this patient to pulmonary hypertension (before and after surgery) and heart failure, hence initial treatment of ACE-I and sildenafil was very necessary.

Brain Abscess Due to Neglected Tetralogy of Fallot: A Case Report

Backgroiund: Tetralogy of Fallot (TOF) is a type of congenital heart disease accounts for about 10% of all congenital cardiac deformities, and is the most common cyanotic lesion after the first year of life. The ideal age for correction of tetralogy of Fallot is still under discussion. Non-cardiac manifestation due to oxygen deficiency has to be aware of in cyanotic patient is brain abscess, because of the right-left shunt in long standing TOF.
 Objectives: To report a case of neglected TOF.
 Case And Discussion: A one-year-old male presented to Murni Teguh Memorial Hospital with the chief complaint of stiffed neck for the past 3 days and got fever for 10 days before admitted, with nausea and vomiting. He was diagnosed with Tetralogy of Fallot through an echocardiography examination when he was 2 months old, but never go through any medical nor surgical treatment. The boy responded to verbal, no eye contact, high grade fever, stiffed neck, Kernig sign and Brudzinski sign was found, and the right extremities were spastic, clubbing finger. On cardiac examination, there was systolic murmur grade ¾ in the left second intercostal space. Echocardiography evaluation revealed dilated RA-RV, no PDA shunt, large mal alignment ventricular septal defect, overriding aorta > 50%, right ventricular hypertrophy, and severe infundibular pulmonary stenosis, consistent with Tetralogy of Fallot. During hospitalization, there were several episodes of seizure and decreased of consciousness, brain CT investigation was done. The abscess was evacuated and a ventriculoperitoneal shunt was performed. One week after the operation, the patient developed abdominal distention, green bile like vomiting, decreased bowel movement and soon muscular defense. From the plain abdominal x-ray and CT revealed peritonitis and intestinal obstruction. An emergency laparotomy was performed, followed by adhesiolysis and jejunostomy due to jejunal perforation. The boy passed away after several episodes of septic shock.
 Conclusions: We would like to emphasis the consequence of the neglected treatment in infant with TOF.

Infundibular pulmonary stenosis and single coronary artery

Infundibular pulmonary stenosis and single coronary artery

Angiotensin converting enzyme inhibitors and angiotensin receptor blockers in an outbreak of SARS-CoV-2

Infundibular pulmonary stenosis is one of the numerous pathologic features of the right tract (from the right ventricular outflow tract to the peripheral pulmonary arteries) occurring at the infundibular level. It is usually associated with ventricular septal defect or part of tetralogy of Fallot, but rarely isolated.

Isolated infundibular pulmonary stenosis. A case report

After a cardiovascular illness, an aviation license becomes invalid until the pilot obtains a special issuance medical certificate from the US Federal Aviation Agency (FAA) affirming successful medical treatment and rehabilitation.

Experience with balloon pulmonary valvuloplasty and predictors of outcome: a ten-year study.

Balloon pulmonary valvuloplasty is the treatment of choice for patients with moderate to severe pulmonary valve stenosis. An observational retrospective cross-sectional study including neonates, small infants, and children who underwent balloon pulmonary valvuloplasty in the period from 2007 to 2016 in the cardiac catheterisation unit of the paediatric cardiology department in Cairo University. Multivariable models were built to report the predictors of the outcome of balloon pulmonary valvuloplasty and its complications. A total of 1200 patients were included in the study and divided according to age into 3 groups: neonates and early infants (n = 282), infants (n = 362), and children (n = 556). Procedural success, defined as a drop pressure gradient across the pulmonary valve to less than or equal to 50% of the baseline measurements, was achieved in 82.7% of the patients. Multivariate analysis revealed that only infundibular pulmonary stenosis (p value 0.032), supravalvular in association with valvular pulmonary stenosis (p value <0.001), and pulmonary valve diameter by angiogram (p value <0.001) were significant predictors of success. The presence of supravalvular in association with valvular pulmonary stenosis (p value <0.001) was associated with a lower weight (p value 0.007) and higher right ventricular pressure before the intervention (p value <0.001), and a minor immediate drop in the pressure gradient post-intervention (p value <0.001) was found to be the most significant predictor of the occurrence of complications. The absence of infundibular and supravalvular stenosis and a large pulmonary valve diameter were the most significant predictors of success.

Echocardiography to Evaluate Pulmonary Stenosis

Congenital pulmonary stenosis (PS) is a common term for lesions causing right ventricular outflow obstruction. It can be further classified as the valvar, supravalvar, and infundibular PS. The PS may often present with other congenital heart diseases. In this article, echo imaging of isolated PS has been discussed. It is imperative to know that the guideline for intervention in isolated PS is totally based on echocardiography. Echocardiographic guidance is required for the selection of procedure, hardwares, evaluation of the outcome of the procedure, and long-term prognosis.

P696 The role of three dimensional echocardiography in a case of recurrent infundibular pulmonary stenosis in a surgically corrected tetralogy of fallot

Abstract Background Tetralogy of Fallot (TOF) represents approximately 7%-10% of congenital heart diseases (CHDs), and it is the most common cyanotic CHD, with 0.23-0.63 cases per 1,000 births. In our case report we are reviewing the added value of three dimensional echocardiography in the assessment of degree of infundibular and valvular stenosis in tetralogy of Fallot. Case report A 29-year-old female patient with history of congenital heart disease (Fallot’s tetrology) and history of total surgical correction at the age of 10 presented to our facility by dyspnea grade III that started 1 month before presentation .On examination; an ejection systolic murmur was heard over the pulmonary area with palpable thrill. Electrocardiogram (ECG) revealed normal sinus rhythm with a heart rate of 75bpm with right axis deviation and right ventricular hypertrophy voltage criteria. Transthoracic two dimensional echocardiography revealed increased thickness of the right ventricle (RV) free wall , and aliasing of the color Doppler flow across the right ventricular outflow tract (RVOT) with a peak systolic gradient across the RVOT 69mmHg . By performing three dimensional transesophageal echocardiography we were able to demonstrate the RVOT narrowing in RVOT enface view and by using specific software we were able to demonstrate the morphology of the pulmonary valve and the pulmonary valve orifice area and we found that the pulmonary valve is a bicuspid valve as shown and the pulmonary valve orifice area was 3.8cm2 signifying the absence of significant valvular stenosis. Conclusion 3D echocardiography in adult congenital heart disease provides unique projections from the living complex anatomy (such as en face views) and true volumetric quantification without geometric assumptions Abstract P696 Figure. Infundibular stenosis

Noonan Syndrome in a 14-year-old Female with Heart Failure

Noonan syndrome is a genetic disorder leading to multiple congenital abnormalities and other health complications, with an incidence of one in 1,000 to 2,500 live births. It is difficult to identify because of its various clinical manifestations. This is the case of a 14-year old female admitted for cardiac failure. The patient was born with normal birth weight but had stunted growth and started walking at the age of five years old. She had speech as well as learning difficulties. She had curly hair, deeply grooved philtrum, ptosis, hypertelorism, micrognathia, low set ears, webbed neck, pectus carinatum, mild thoracic scoliosis and short stature. Her bone age was in line with that of an 8–10 months old infant. Her luteinizing hormone level was below normal. Echocardiography showed severe infundibular pulmonary stenosis, secundum atrial septal defect (ASD), bidirectional shunt with a diameter of 2.1 cm, an anterior rim of 3.71 cm and a posterior rim of 1.17 cm. She also had severe tricuspid regurgitation due to severe infundibular pulmonary stenosis, and moderate mitral regurgitation due to posterior mitral leaflet prolapse. She received hormonal therapy, an angiotensin-converting enzyme inhibitor, a beta-blocker, spironolactone and furosemide. Resection of the infundibular muscle, ASD closure and repair mitral valve was planned. KEYWORDS: diagnosis, management, Noonan syndrome

Isolated Infundibular Pulmonary Stenosis with Infectious Endocarditis: a Rare Ecocardiographic Finding

Isolated Infundibular Pulmonary Stenosis with Infectious Endocarditis: a Rare Ecocardiographic Finding

Isolated Infundibular Pulmonary Stenosis in Adult

Isolated infundibula pulmonary stenosis (UPS) in adult is an uncommon cardiac abnormality. Infundibula pulmonary stenosis leading to right ventricular hypertrophy is a big problem. Early excision of hypertrophied muscular band can overcome this problem. In our case report we have done excision of pulmonary infundibula muscle band of a late case of isolated pulmonary infundibula stenosis. And our patient showed a good response after excision of infundibula muscle band.University Heart Journal Vol. 13, No. 2, July 2017; 65-66