Mild Pulmonary Valve Stenosis Research Articles

A Pediatric Cardiology Outreach Clinic in a Local Hospital: An Experience Review from South A'Sharqiyah, Oman.

Specialized pediatric cardiology clinics conducted in local hospitals are an important part of delivering specialized care to patients close to their homes. This study aimed to review our experience with a specialized pediatric cardiology outreach clinic at Jaalan Bani Bu Ali Hospital, South A'Sharqiyah, Oman. Patient records for each individual, seen in the outreach clinic between March 2018 and June 2022, were reviewed to determine demographic information, reason for referral, underlying diagnosis, and clinic visit outcomes. Over the study period, 29 clinics were conducted, with 360 patients seen. Of these, 200 (55.6%) were male with a median age of 13 months. The majority of patients (n = 271; 75.3%) were referred due to a cardiac murmur. Most patients had a normal cardiac evaluation (n = 177; 49.2%). The most common congenital heart diseases detected were mild pulmonary valve stenosis (14.8%) and moderate to large secundum atrial septal defects (13.7%). Significant cardiac lesions detected included severe pulmonary hypertension (2.2%), tetralogy of Fallot (1.6%), and cor triatriatum sinistrum (0.5%). Overall, 70 (19.4%) patients were referred to tertiary care hospitals, and 179 (49.7%) were reassured and discharged. Conducting specialized pediatric cardiology outreach clinics in overpopulated areas is effective and well-received by families. It reassures many families and reduces the need for unnecessary travel to specialized centers. These clinics also play a crucial role in detecting patients with significant cardiac defects requiring urgent care. Implementing specialized clinics in primary and secondary health centers could be beneficial for other subspecialties in reducing long waiting lists.

Whole exome sequencing of a novel homozygous missense variant in PALB2 gene leading to Fanconi anaemia complementation group.

Partner and localiser of BRCA2 (PALB2), also known as FANCN, is a key tumour suppressor gene in maintaining genome integrity. Monoallelic mutations of PALB2 are associated with breast and overian cancers, while bi-allelic mutations cause Fanconi anaemia (FA). In the present study, whole exome sequencing (WES) identified a novel homozygous missense variant, NM_024675.3: c.3296C>G (p.Thr1099Arg) in PALB2 gene (OMIM: 610355) that caused FA with mild pulmonary valve stenosis and dysmorphic and atypical features, including lymphangiectasia, non-immune hydrops fetalis and right-sided pleural effusion in a preterm female baby. WES results were further validated by Sanger sequencing. WES improves the screening and detection of novel and causative genetic variants to improve management of disease. To the best of our knowledge, the present study is the first reported FA case in a Saudi family with phenotypic atypical FA features. The results support the role of PALB2 gene and pathogenic variants that may cause clinical presentation of FA. Furthermore, the present results may establish a disease database, providing a groundwork for understanding the key genomic regions to control diseases resulting from consanguinity.

Analysis of echocardiographic features and postnatal management of fetal pulmonary valve stenosis

Objective: To investigate the echocardiographic features, consistency of diagnosis between fetal and postnatal periods and postnatal clinical outcomes of fetal pulmonary valve stenosis (PS) with different degrees. Methods: This study was a retrospective cohort study comprising 108 cases of fetal PS diagnosed during the fetal period and followed up postnatally at Xinhua Hospital, Shanghai Jiaotong University School of Medicine from November 2012 to February 2023. Echocardiographic characteristics, including morphological and hemodynamic features were collected for all fetuses who were then were followed up to at least 6 months after birth. One-way analysis of variance and Kruskal-Wallis test were used to compare the differences in the echocardiographic features among fetuses with different degrees of PS. Subsequently, McNemar test was used to assess the consistency of diagnosis between the fetal and postnatal periods. Furthermore, Logistic regression analysis was applied to explore the risk factors for neonatal intervention in fetuses with moderate PS and the receiver operating characteristic (ROC) curve was utilized to ascertain the optimal cut-off value for continuous variables. Results: The age of the mothers of the 108 fetuses at the initial assessment was (30.8±4.0) years, and the gestational age was 26.5 (24.6, 30.0) weeks. The fetuses were categorized into mild (17 cases), moderate (49 cases), and severe groups (42 cases) based on the initial echocardiographic features. Mild PS was characterized by valve thickening and hyperechogenicity combined with systolic flow acceleration or dilation of main pulmonary artery. Moderate PS exhibited both restricted valve motion and a colorful blood flow pattern at the valve orifice. The peak flow velocities of fetuses with moderate and critical PS were notably higher than those in the mild group ((2.66±0.86) and (2.77±1.30) vs. (1.43±0.59)m/s, F=14.52, P<0.001). In critical PS, all cases showed retrograde ductal flow, with a significantly higher proportion of a small right ventricle compared to the mild and moderate PS (42.9% (18/42) vs. 0 and 2.0% (1/49), χ2=31.73, P<0.001). The proportion of severe tricuspid regurgitation was also higher (35.7% (15/42) vs. 0 and 10.2% (5/49), χ2=36.94, P<0.001). Compared to mild and severe PS, the consistency of diagnosis between fetal and postnatal periods in moderate PS was lower (40.8% (20/49) vs.13/17 and 80.3% (35/42), χ2=12.45, P=0.006). The systolic flow velocity was identified as an independent risk factor for neonatal intervention in fetuses with moderate PS (OR=7.21, 95%CI2.11-24.62). A flow velocity of ≥2.18 m/s in second trimester and ≥3.15 m/s in third trimester indicated the necessity of neonatal intervention for fetal moderate PS. Among the 108 fetuses, 68 underwent surgical intervention and all survived. Additionally, 39 fetuses were regularly followed up. A sole non-surgical fatality occurred, leading to a 6-month survival rate of 99.1% (107/108). Conclusions: Various degrees of fetal PS demonstrate distinctive morphological and hemodynamic alterations in echocardiography. The disparity in severity between the postnatal and fetal stages requires ongoing monitoring for fetal PS. The prognosis for fetal PS is generally favorable.

Complexity of dental management of a pediatric patient with lamellar ichthyosis: case report

The purpose of this case report is to describe the complexity of dental management of a pediatric patient with lamellar ichthyosis. A 9-year-old male patient was referred by the pediatrician to the special needs’ clinic with a major complaint of "tooth in the roof of the mouth", according to the person in charge. The patient's mother reported that he was diagnosed with lamellar ichthyosis at birth and regularly followed up with a medical team, including a cardiologist, due to the presence of mild pulmonary valve stenosis. The physical examination revealed presence of skin peeling, everted lip, mouth breathing, cognitive deficit, and speech delay. The intraoral clinical examination showed mixed dentition, absence of caries lesions and presence of supernumerary teeth on the palate, between teeth 11 and 21, which was confirmed by radiographic examination. Although he was cleared by the pediatrician and cardiologist for the procedure under general anesthesia, this procedure in the hospital setting was interrupted at the last minute and contraindicated by the anesthesiologist, as it would be a high risky due to the patient's airway conditions. Thus, one month later, the patient's surgery was performed under local anesthesia on an outpatient basis under sedation and protective stabilization. The patient remains on follow-up and receiving preventive treatment. Thus, it is fundamental that there is a multidisciplinary approach of these patients to prevent possible complications resulting from dental procedures. In addition, regular dental monitoring is essential to avoid subjecting the patient to invasive procedures.

959 AN UNEXPECTED DIAGNOSIS BY ECHOCARDIOGRAPHY: APPLICATION IN CLINICAL PRACTICE

Abstract A carcinoid tumore is a rare neuroendocrinologic malignancy that commonly origins from enterochromaffin or Kulchitsky cells in the gastrointestinal tract and secretes bioactive molecules in blood. Up to 60% of patients with carcinoid tumors have cardiac involvement, often on the right-sided heart valves (typically the tricuspid valve and the pulmonic valve). A 66 year old woman was admitted to hospital with congestive heart failure, chronic diarrhea and weight loss. A contrast-enhanced computed tomography scan of the liver showed multiple masses (the largest 70 mm×45mm) compatible with metastases. A transthoracic echocardiogram showed dilatation of right sections of the heart with severe tricuspid regurgitation (RV 64 ml/beat, ERO 1 cmq) and mild pulmonary valve stenosis (Peak gradient 13 mmHg). Patient's aortic and mitral valves were normal in structure and function. The EKG revealed low-voltage QRS complexes of all leads, unspecific ST segment changes and sinusal rhythm. The blood tests showed a significant increase in chromogranin A (17670 ng/ml) and enolase; a 24-hour urine collection showed a total output of 5-hydroxyindoleacetic acid eleveted. A biopsy of the liver with immunostaining for chromogranin and synaptophysin were strongly positive and confirmed the diagnosis of metastatic carcinoid syndrome (grade 1, cells CDX2 positive). The echocardiographic findings of carcinoid heart disease is drumstick-like, rigid, and shortened leaflets with a visible regurgitant orifice, causing torrential tricuspid regurgitation. The pulmonic valve presents more frequently valvular stenosis. A combination of biomarkers and cardiac imaging is useful in screening and diagnosis of carcinoid heart involvement in patients with carcinoid syndrome. The management of carcinoid heart disease is hardful and requires a multidisciplinary approach, medical and surgical treatment, for optimized care.

Biventricular impact of mild to moderate fetal pulmonary valve stenosis.

To define the pattern of fetal echocardiographic changes associated with isolated pulmonary valve stenosis (PS) and to correlate the echocardiographic findings with neonatal outcome and the need for postnatal pulmonary valvuloplasty within the first 12 months postpartum. This was a prospective cohort study between January 2009 and October 2015 of 16 fetuses with isolated PS and 48 controls matched by gestational age at ultrasound examination (± 2 weeks) evaluated at the Fetal Cardiology Unit at BCNatal (Barcelona). Standard fetal ultrasound and comprehensive echocardiography, which included cardiovascular morphometric parameters, and systolic and diastolic functional and timing measurements, were performed in all cases. Baseline characteristics and perinatal outcome were retrieved from clinical records. Cases were followed up until 12 months of age, and admission to intensive care unit, days of hospitalization, need for prostaglandins and requirement for postnatal surgery were reviewed. Fetal PS cases were analyzed according to the need for postnatal pulmonary valvuloplasty. The study groups were similar in terms of baseline, fetal ultrasound and perinatal characteristics. Median gestational age at diagnosis of PS was 33.4 (range, 20.0-36.5) weeks. Most cases corresponded to mild or moderate PS; only three fetuses had reversed flow in the ductus arteriosus before delivery. Six (37.5%) newborns, including all three with reversed flow in the ductus arteriosus prenatally, required postnatal pulmonary valvuloplasty. Fetuses with PS presented with larger and more globular hearts, with increased myocardial wall thickness in the third trimester. Despite preserved right ventricular (RV) ejection fraction and systolic longitudinal motion, PS cases showed increased right cardiac output and signs of diastolic dysfunction, with higher ductus venosus pulsatility index (0.72 ± 0.32 vs 0.53 ± 0.16, P= 0.004) and tricuspid E/E' ratio (7.52 ± 3.07 vs 5.76 ± 1.79, P= 0.022). In addition, fetuses with PS displayed a compensatory increase in left ventricular (LV) radial and longitudinal motion, as shown by a higher ejection fraction (79.3 ± 8.23% vs 67.6 ± 11.3%, P= 0.003) and mitral annular-plane systolic excursion (5.94 ± 1.38 vs 5.0 ± 1.22 mm, P= 0.035). Finally, fetuses requiring postnatal pulmonary valvuloplasty showed a different pattern of echocardiographic findings from those not requiring valvuloplasty, with a significantly smaller RV and pulmonary valve diameter, reduced tricuspid annular-plane systolic excursion (5.08 ± 1.59 vs 8.07 ± 1.93 mm, P= 0.028), increased LV cardiac output (340 ± 16 vs 176 ± 44 mL/min/kg, P =0.003) and more pronounced signs of LV diastolic dysfunction (mitral E' velocity, 5.78 ± 0.90 vs 8.16 ± 1.58 cm/s, P= 0.008). Fetuses with PS present with more hypertrophic, larger and more globular hearts in the third trimester of pregnancy, associated with a higher right cardiac output and impaired biventricular relaxation. In addition, signs of increased LV contraction were observed. Our data suggest that RV and LV functional parameters could be useful for predicting the need for postnatal pulmonary valvuloplasty. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Video-Assisted Thoracoscopic Thymectomy Langerhans Cell Histiocytosis

Introduction: Langerhans cell histiocytosis (LCH) is an exceedingly rare proliferative disorder in which pathologic histiocytic cells accumulate in nearly every organ. Although the deposited cells share morphology and antigens with the skin Langerhans cell, the cell of origin is actually a myeloid dendritic cell. LCH is most common in the pediatric population from ages 1 to 3 effecting ~1–2 patients per million. The most common manifestation of the disease is lytic bone lesions accounting for 77% of those diagnosed. Lymph nodes and the lung are involved in 19% and 10% of cases, respectively. Methods: Our patient, a 5-month-old, 6-kg female with mild pulmonary valve stenosis, had both thymic and lung tissue involvement. To date, there has never been a report of a thymic LCH with lung metastases in an infant. Results: She underwent an echocardiogram to assess the degree of her pulmonary stenosis, which incidentally showed a large thymic cyst. That cyst was further characterized with CT scan as a heterogenous mass with multiple lung nodules. The patient was meeting all milestones appropriately and asymptomatic without cardiac symptoms, respiratory difficulty, or feeding issues. To rule out a thymoma, the patient underwent right video-assisted thoracoscopic thymectomy with wedge biopsy of the right lower lobe of the lung. Conclusion: Final pathology report showed LCH in both tissue samples. The patient did well without complications and was discharged home expeditiously. She is now under the care of the medical oncologists for continued management. No competing financial interests exist. Runtime of video: 4 mins 30 secs

Absent pulmonary valve syndrome with left lung atresia.

Absent pulmonary valve syndrome (APVS) is a rare anomaly characterized by the absence of the pulmonary valve. The absence of the pulmonary valve, along with the hypoplasia of the pulmonary annulus, leads to aneurysmal dilatation of the pulmonary artery and its branches. Other consequences include pulmonary stenosis and pulmonary insufficiency (1). The 3-day-old baby presented with cyanosis and tachypnea. He had to and fro murmurs at the upper left sternal region, as well as hepatomegaly. The electrocardiogram showed right ventricular hypertrophy. The posterior-anterior chest X-ray showed opaque left hemithorax with cardiac displacement (Figure 1). Upon echocardiographic examination, the following findings were noted: the right cardiac chambers were dilated and left ventricular hypoplasia was present (Video 1. See corresponding video/movie images at www.balkanmedicaljournal.org). A large subaortic ventricular septal defect and a main pulmonary artery aneurysm were seen (Video 2. See corresponding video/movie images at www.balkanmedicaljournal.org). Hypoplastic pulmonary annulus and absent pulmonary valve leaflets were present (Figure 2). Moderate pulmonary stenosis (peak systolic gradient was 45 mmHg) and mild pulmonary regurgitation were present (Video 3. See corresponding video/movie images at www.balkanmedical-journal.org). The aortic arc was left sided and no patent ductus arteriosus was seen. In addition to the echocardiographic findings, atresia of the left pulmonary artery was observed with computed tomography (Figures 3, ​,4).4). The patient died due to respiratory failure on fourth day after birth. FIG. 1 Posterior-anterior chest X-ray showed opaque left hemithorax with cardiac displacement FIG. 2 Hypoplastic pulmonary annulus and absent pulmonary valve leaflets FIG. 3 CT 3D reconstruction of aneurysmal pulmonary arteries FIG. 4 Left pulmonary artery atresia Absent pulmonary valve syndrome is a rare anomaly, which is characterized by aneurysmal dilatation of the pulmonary artery, depending on atresia or dysplasia of the pulmonary valve leaflets. The disease is usually associated with tetralogy of Fallot. Due to the absence of pulmonary valve leaflets, pulmonary regurgitation and mild pulmonary stenosis were seen (2). Respiratory distress signs could be seen to varying degrees due to compression of the airway with the pulmonary artery. In the severe forms, due to complete obstruction of the trachea or bronchi, rapid progress can be seen in the neonatal period. More severe forms of the disease may result in death during the newborn period due to the pressure effect exerted by the pulmonary artery on the tracheobronchial structures. The patient presented herein had an absence of the pulmonary valve, together with the non-restrictive ventricular septal defect. Also, mild pulmonary valve stenosis and insufficiency, widened pulmonary artery, and atresia of the left pulmonary artery were seen. Although catheterization could not be performed during the early stage, as the patient had severe respiratory dysfunction, computed tomography confirmed the presence of a widened pulmonary artery and atresia of the left pulmonary artery. Also, arterial branches from the abdominal or thoracic aorta supplying the left lung could not be detected with computed tomography. The combination of APVS with atresia of the left pulmonary artery is extremely rare (3). Early diagnosis and supportive treatment in the newborn period are important for the survival of these severely affected infants.

Incidence of congenital heart disease in Palestinian children born in the Gaza Strip, occupied Palestinian territory: a cross-sectional study

Background Congenital heart disease is the most frequent form of congenital anomaly in newborn infants, and accounts for more than a quarter of all serious congenital anomalies worldwide. Generally, an estimated incidence of eight cases of congenital heart disease per 1000 livebirths is internationally accepted as the best approximation. The causes of most congenital heart defects are not known. This study was designed to estimate the birth incidence of children with congenital heart disease born in the Gaza Strip, occupied Palestinian territory, during 2010, and to compare this estimate with estimates from west Europe, North America, and Asia. Methods We reviewed the medical records of all children born in 2010 who were diagnosed, treated, or followed up in the four paediatric cardiology clinics in the Gaza Strip. Data were also obtained from other medical centres to which some of our patients were referred for percutaneous or surgical treatment. Patients were excluded if they had isolated patent foramen ovale; rhythm disturbances without structural defects; isolated mild peripheral pulmonary stenosis; isolated innocent patent ductus arteriosus; patent ductus arteriosus (preterm infants); hereditary disorders without cardiac consequences; or malpositioning of the heart without structural defects. Systematic echocardiographical screening of the entire population is not done, so subclinical heart defects, such as subclinical ventricular septal defect, mild pulmonary valve stenosis, or small atrial septal defect will have been missed. Babies who died perinatally or after birth before diagnosis in the Gaza Strip are not examined at post mortem. The study was approved by the human research ethics committee, Ministry of Health, Gaza Strip. Data were analysed with Microsoft Excel 2007. Because existing data were used, informed consent was required from the parents or guardians of the children. Findings Congenital heart disease was detected in 598 of 59 757 children who were born alive in the Gaza Strip during 2010, giving an incidence of ten per 1000 livebirths. 334 (56%) of 598 children were girls and 264 (44%) were boys. The most frequent anomalies were ventricular septal defects (167 [28%]), ostium secundum atrial septal defects (101 [17%]), patent ductus arteriosus (52 [9%]), and pulmonary valve abnormalities (48 [8%]). 150 children (25%) had a cardiosurgical operation and 48 (8%) had a catheter intervention. 42 (7%) children died. At ages 6 months and 1 year, 562 (94%) and 556 (93%) children were alive, respectively, and remained stable during 18 months of follow-up. Interpretation The results of this study are relevant not only for clinicians and hospital administrators, but also for public health in the Gaza Strip. Furthermore, the findings can be used for a longitudinal follow-up of the cohort of patients in this study to ascertain progress and outcomes of patient management. Funding None.

Early Double Switch With Lecompte Maneuver for Life-Threatening Airway Obstruction

A 6-month-old boy with congenitally corrected transposition of the great arteries, ventricular septal defect, and mild pulmonary valve stenosis presented with acute obstructive respiratory life-threatening events. Airways obstruction was caused by aneurysmal dilation of the branch pulmonary arteries compressing the carina and bronchi. The child was ventilator dependent. A double switch operation with Lecompte maneuver was performed, bringing the pulmonary arteries forward and relieving the airway compression. The child made a complete recovery and remains well 9 months later with no clinical or radiologic signs of airway compression.

症例 著明な肺動脈拡張をきたした無症候性の部分肺静脈還流異常,心房中隔欠損および軽度肺動脈弁狭窄の合併例

症例 著明な肺動脈拡張をきたした無症候性の部分肺静脈還流異常,心房中隔欠損および軽度肺動脈弁狭窄の合併例

Aneurysm of pulmonary artery

A 21-year-old female previously diagnosed as having mild pulmonary valve stenosis (PS) presented with breathlessness, reduced exercise tolerance, intermittent palpitations and chest pain. There were no cardiovascular risk factors or...

"Vanishing" pulmonary valve stenosis

Objective:Both spontaneous resolution and progression of mild pulmonary valve stenosis (PS) have been reported. We reviewed characteristics of the pulmonary valve (PV) to determine factors that could influence resolution of mild PS.Methods:Fifteen asymptomatic pediatric patients with spontaneous resolution of isolated mild PS were retrospectively reviewed.Results:There was no correlation between the PV gradient, clinical presentation, age at diagnosis, or PV morphology. The PV annulus was small at initial presentation, which normalized at follow up. When corrected for the body surface area (z-score), the PV annulus was normal in all patients, including at initial evaluation.Conclusions:Based on our observation, neither age at diagnosis, nor PV-morphology-influenced resolution of mild PS. The variable clinical presentation makes it difficult to categorize and observe mild PS by auscultation alone. The PV annulus z-score could be a useful adjunct to determine the course and serial observation of mild PS.

Ovarian Tumor-Associated Carcinoid Heart Disease Presenting as Severe Tricuspid Regurgitation

Carcinoid heart disease is characterized by heart valve dysfunction as well as carcinoid symptomatology. We report a case of carcinoid heart disease associated with a primary ovarian tumor. A 60-year-old woman presented for dyspnea evaluation with a history of facial flushing, telangiectatic skin changes, and pitting edema of both lower extremities. Chest radiography showed cardiomegaly, and echocardiography revealed an isolated, severe tricuspid regurgitation without left-sided valvular dysfunction. The tricuspid leaflets were severely retracted and shortened, resulting in poor coaptation. Furthermore, mild pulmonary valve stenosis and moderate regurgitation were found along with this deformation. The 24-hour urine analysis revealed an increased level of 5-hydroxyindoleacetic acid, and an ovarian tumor was apparent on computed tomography images. The mass was surgically removed, and the patient was diagnosed as having a primary ovarian carcinoid tumor. She was treated with chemotherapy and regularly followed-up with supportive treatments, deferring surgical correction.

A Management Strategy for Mild Valvar Pulmonary Stenosis

Pulmonary valve stenosis (PVS) is a common congenital cardiac lesion, 1/1000 live-births, the majority of patients having mild transvalvar gradients. In the present study, we sought to determine the outcome of mild PVS diagnosed by echocardiography and to propose a management algorithm that would identify patients at risk for progression of PVS, yet reduce health care expenditures. In this single-center retrospective study, we included all patients who met the following criteria: first diagnosed with PVS at <10 years of age, an initial peak systolic Doppler gradient of < or =40 mm Hg, no additional congenital heart lesions, and at least two clinical evaluations. There were 146 subjects who met these criteria. The median age at initial diagnosis was 3.9 months, with a range of 1 day to 9.9 years. The average initial peak systolic gradient (PSG) was 23.3 mm Hg (+/-7.8) and final PSG 17.1 mm Hg (+/-10.3). Over a mean length of follow-up of 4.0 years, 107/146 (73%) were later reported to have very mild PVS (PSG < or = 25 mm Hg, no clinical change or resolution of murmur). Only 3 of 146 subjects progressed to have a PSG above 40 mm Hg, with 1 undergoing a balloon valvuloplasty. In conclusion, mild PVS diagnosed in early childhood is a benign lesion, with most children essentially demonstrating resolution in the first years of life. Mild PVS identified in infancy requires a brief period of close observation. Based on these data, our management recommendations are that infants be followed closely within the first year of life. Young infants diagnosed at birth that maintain a PSG < or = 25 mm Hg at more than 6 months of age as well as those who are older than 1 year of age with a PSG < or = 40 mm Hg have a benign course and the utility of ongoing cardiology follow-up is questionable.

Mild pulmonary valve stenosis: the possible spontaneous cure in the natural history of the defect

To determine the long-term natural history of patients with mild pulmonary valve stenosis. Throughout a 24-year evolutive period (1980 to 2004), 83 patients with pulmonary valve stenosis were evaluated, with 29 of them presenting a mild form of the defect. Of these 29, 13 patients had a long-term follow up. The mean age at the first and last follow-up visits was 34 months (1 month to 15 years) and 10.5 years (3 to 24 years), respectively. In addition to evolutive elements, those regarding clinical, electrocardiographic and echocardiographic aspects were also assessed. All patients remained asymptomatic throughout the study. Regarding the pulmonary valve pressure gradient, 5 (38.4%) presented a decrease, 3 (23%) remained unchanged, 4 (30.7%) presented elimination and 1 (7.6%) presented accentuation to a moderate degree of involvement. The mean initial and final gradient was 24.2 mmHg (15-30) and 13.6 mmHg (0-54), respectively. The initial gradients of the 4 patients who achieved spontaneous cure were 30, 19, 30 and 20 mmHg. The systolic murmur and right ventricular overload had a direct correlation to the gradient pressure degree, subdivided at each 10 mmHg. The spontaneous cure of the mild pulmonary valve stenosis is possible to achieve, similarly to other acyanogenic anomalies.

Late cardiac effects of mediastinal radiotherapy in patients with Hodgkin's disease.

Twenty-eight patients younger than age 40 years, treated for Hodgkin's disease with mediastinal irradiation, were examined no less than 5 years after the irradiation in order to evaluate the frequency of cardiac abnormalities. Twelve patients (43%) had had some pericardial event after radiation: a diagnosed pericarditis, remarkably increased heart volume, or a conspicuous change of cardiac silhouette, suggesting pericardial fluid. On evaluation, 50% of the patients complained of symptoms, and 13 patients had to stop the exercise test on a low level because of chest pain, dyspnea, or general fatigue. In 13 patients some of the following abnormalities in the electrocardiogram (ECG) was found: right bundle branch block (four), first-degree atrioventricular block (four), abnormal P terminal force (five), or a low voltage (two). In ten patients (38%) an increase of the pericardial fluid was seen in the echocardiogram, and in nine patients the right ventricle wall thickness had increased. In two patients a severe coronary artery disease was found. One died suddenly after an acute myocardial infarction (AMI), and the other had a large anterior AMI. Two patients with chronic pericardial fluid underwent partial pericardectomy. Two cases of mild pulmonary valve stenosis, one pulmonary subvalvular stenosis and two aortic valve deformities were discovered. Eight patients went through cardiac catheterization, and in all but one case the pressures were slightly elevated suggesting diminished diastolic compliance. In summary, 19 of 28 patients had some abnormal cardiac findings, but only three of them were serious ones.

Upright T wave in V 1: A useful diagnostic sign of mild pulmonic valve stenosis in children

In 9 of 10 children aged 5 to 10 years with mild pulmonary value stenosis, confirmed at cardiac catheterization and cineangiocardiography, the T wave in V 1 was upright in an otherwise normal electrocardiogram. This compared with absence of an upright T wave concluded that the presence of an upright T wave in V 1 in children with a systolic in V 1 in 6 cases of atrial septal defect and in 100 cases of functional murmurs. It is murmur and an otherwise normal electrocardiogram is strongly suggestive of mild pulmonary valve stenosis.

Spontaneous physiologic closure of an atrial septal defect after infancy ∗

The rare occurrence of spontaneous physiologic closure of an atrial septal defect in an older child was reported. Associated lesions were mild pulmonary valve stenosis and a right aortic arch. Closure of the defect was documented by two right heart catheterizations and selective angiocardiography in the left atrium. The exact incidence, as well as mechanism of closure, is unknown.