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Background The significance of depression/anxiety among ACHD patients in terms of health care utilization is unknown and data on the association with mortality are scarce. Methods Analyses comprised 8334 ACHD patients, age ≥ 18 years, insured by a large healthcare organization (2007–2011). Depression/anxiety were determined by diagnoses and treatments recorded in the organization database. Adjusted utilization relative rates (RRs) were estimated with negative binomial models and mortality hazard ratios (HRs) with the Cox proportional hazard model. Results ACHD patients with depression/anxiety (N = 2950, 35%) were more likely to be older (mean ± SD: 54 ± 17 vs. 45 ± 18 years), women (61% vs. 45%), and have comorbidities than counterparts without depression/anxiety. Following multivariable adjustment, patients with depression/anxiety had more primary care and cardiology clinic visits, more emergency department visits and more hospitalizations. RRs (95% confidence interval) were: 1.31 (1.27–1.35); 1.07 (1.01–1.13); 1.60 (1.46–1.77); and 1.18 (1.08–1.29) respectively, for diagnosis before the study period, and 1.36 (1.31–1.42); 1.22 (1.14–1.30); 1.43 (1.24–1.60) and 1.47 (1.33–1.64), respectively, for diagnosis during the study. Stratifying by age, the highest adjusted primary care and cardiology visit RRs were found among 18–24 years old patients and the lowest among patients ≥65 years. Between 2007 and 2017, 905 patients died. Depression/anxiety were associated with increased mortality risk with adjusted HRs: 1.10 (95% CI: 0.94–1.29) for past diagnosis and 1.40 (1.17–1.67) for study period depression/anxiety diagnosis. Conclusions Depression/anxiety in ACHD patients is associated with increased health-care utilization and a higher risk of death. The efficacy of addressing patients' psychosocial needs in optimizing health-care utilization and improving prognosis needs further evaluation.

Background Several studies have examined hospitalizations among patients with adult congenital heart disease (ACHD). Few investigated other services or utilization patterns. Our aim was to study service utilization patterns and predictors among patients with ACHD. Methods and Results We identified 11 653 patients with ACHD aged ≥18 years (median, 47 years), through electronic records of 2 large Israeli healthcare providers (2007–2011). The association between patient, disease, and sociogeographic characteristics and healthcare resource utilization were modeled as recurrent events accounting for the competing death risk. Patients with ACHD had high healthcare utilization rates compared with the general population. The highest standardized service utilization ratios (SSRs) were found among patients with complex congenital heart disease including primary care visits (SSR, 1.53; 95% CI, 1.47–1.58), cardiology outpatient visits (SSR, 5.17; 95% CI, 4.69–5.64), hospitalizations (SSR, 6.68; 95% CI, 5.82–7.54), and days in hospital (SSR, 15.37; 95% CI, 14.61–16.12). Adjusted resource utilization hazard increased with increasing lesion complexity. Hazard ratios (HRs) for complex versus simple disease were: primary care (HR, 1.14; 95% CI, 1.06–1.23); cardiology outpatient visits (HR, 1.40; 95% CI, 1.24–1.59); emergency department visits (HR, 1.19; 95% CI, 1.02–1.39); and hospitalizations (HR, 1.75; 95% CI, 1.49–2.05). Effects attenuated with age for cardiology outpatient visits and hospitalizations and increased for emergency department visits. Female sex, geographic periphery, and ethnic minority were associated with more primary care visits, and female sex (HR versus men, 0.89 [95% CI, 0.84–0.94]) and periphery (HR, 0.72 [95% CI, 0.58–0.90] for very peripheral versus very central) were associated with fewer cardiology visits. Arab minority patients also had high hospitalization rates compared with the majority group of Jewish or other patients. Conclusions Healthcare utilization rates were high among patients with ACHD. Female sex, geographic periphery, and ethnicity were associated with less optimal service utilization patterns. Further research should examine strategies to optimize service utilization in these groups.

Velocardiofacial syndrome (VCFS) is a relatively common developmental neuropsychiatric syndrome caused by a 22q11 microdeletion. There is an extensive variability in the phenotypic expression of this disease. The most common psychiatric disorder in VCFS is attention-deficit/hyperactivity disorder (ADHD), affecting 35-55% of patients. This study investigated the association of familial, developmental, and physical factors with the occurrence of ADHD in 51 patients with nonfamilial VCFS. Twenty-one patients (41.2%) were diagnosed with ADHD. There was a significantly greater prevalence of ADHD in the first-degree relatives of the patients with ADHD than in those without (OR = 5.9, 95% CI = 1.6-22.1, P = 0.006). No differences were noted between the ADHD and non-ADHD groups in mean Obstetric Complication Scale Score, gestational age, birth weight, age at first words, walking, and achieving bowel control. The two groups also had similar IQ scores (total, verbal, and performance) and had a similar average degree of severity of facial dysmorphism and cardiac and cleft anomalies. These findings indicate that ADHD in VCFS has a genetic contribution and the patients' VCFS-related developmental factors and physical illnesses play a lesser role.

Percutaneous pulmonic valve and pulmonic stent implantation have become a well-established treatment for recurrent pulmonic stenosis or insufficiency in patients with repaired congenital heart disease. Late endocarditis is seldom reported, but its diagnosis might be challenging due to the limited visualization of the stented valve or stent by transesophageal echocardiography. We present 2 young patients who were hospitalized for suspected endocarditis and in whom the diagnosis was made with the aid of positron emission tomography/computed tomography scan.

Atrial natriuretic peptide (ANP) is one of the cardiac peptides implicated in volume and sodium homeostasis. We investigated the effect of interventional catheterization on plasma levels of ANP, aldosterone, and cortisol in 28 children with various congenital heart defects (CHD). Patients were divided by age into two groups: group A--infants and children over 3 months of age (n = 22), and group B--newborns (n = 6). These were compared to age-matched control groups. In group A, interventions included pulmonic valvotomy (n = 8), aortic valvotomy (n = 4), balloon angioplasty of native coarctation of the aorta (n = 3), balloon dilatation of the mitral valve (n = 1), and Rashkind double umbrella closure of patent ductus arteriosus (n = 6). Group B interventions included pulmonic valvotomy (n = 3), aortic valvotomy (n = 1), and balloon atrial septosomy (n = 2). In group A, mean ANP levels were markedly higher than in age-matched controls (125.2+/-15.8 vs. 24.6+/-4.6 pg/ml) (P

In recent years, the percutaneous closure of small and medium-sized patent arterial ducts has been achieved using occluding spring coils. We describe our experience in 33 patients with this technique using a snare to facilitate the procedure. All patients had a clinically apparent patent arterial duct and underwent an attempt at transcatheter closure at a mean age of 5.6 years. In one patient, the duct was a residual lesion following surgical ligation, and in three was residual following attempted closure with a Rashkind double-umbrella. The mean narrowest diameter of the ducts was 1.9 mm. The coil embolized in two of the first four patients, and subsequent to that experience we used a snare to improve delivery. Of the 33 patients, implantation was successful in 32 (97%) using one [29 patients] or two [two patients] coils and in one by a combination of a double-umbrella device and an occluding spring coil. The mean fluoroscopic screening time for the whole group was 26.5 minutes, this time decreasing to 18.5 minutes in the last 26 patients [p

Pulmonary venous pathway obstruction developed after a Mustard procedure in an infant with transposition of the great arteries. The stenosis was successfully dilated by the percutaneous balloon technique with immediate clinical, echocardiographic, and hemo-dynamic improvement, which has persisted for 8 months after dilation. This technique is a feasible alternative to reoperation in such an obstruction, even in infants.

In 63 patients with various congenital heart defects, lung perfusion was evaluated with technetium-99mm macroaggregated albumin. Right lung perfusion abnormalities were documented in 34 patients (54%). A particularly high incidence occurred in patients who had undergone a systemic to pulmonary artery shunt operation as an initial palliative procedure or who had had right ventricular outflow reconstruction and in those with bilateral pulmonary artery stenosis. Serial studies were helpful in evaluating the functional results of different transcatheter interventions for optimizing pulmonary blood flow. The quantitative relative perfusion radionuclide method was a more sensitive means of detecting cases of abnormal lung perfusion than was chest radiology.

Fifteen patients aged 10 to 18 years with syncope of unknown origin, and 10 healthy control children aged 11 to 18 years, were evaluated by head-up tilt to 60 degrees for 60 minutes. Six patients (43%) reproduced symptoms of syncope during the examination. Four had a typical vasovagal reaction; two had marked hyperventilation. None of the children in the control group had syncope. The head-up tilt test offers a simple, noninvasive, high-yielding diagnostic tool for evaluation of syncope in children.

This case demonstrates a novel use of stent implantation for relief of coarctation of the aorta caused by protrusion of a Rashkind patent ductus arteriosus umbrella. Follow-up 3 years after stent implantation shows complete relief of obstruction.

To the Editors: We read with interest the important article by Jais et al . [1] in a recent issue of the European Respiratory Journal discussing management and outcome of pregnancy in pulmonary hypertension patients in the light of recent advanced therapy. We certainly agree that this issue should be revisited in the current era when many new potent medications are available. Yet, even in the current era with optimal management, this condition carries substantial risks and demands careful examination of the data …

Background. An accurate assessment of fluid status in haemodialysis patients presents a significant challenge especially in growing children. Clinical parameters of hydration are not always reliable, and invasive methods such as measurement of central venous pressure cannot be used routinely. We evaluated the usefulness of inferior vena cava diameter (IVCD) measured by echocardiography in the estimation of hydration in children on haemodialysis. Methods. Fifteen haemodialysis patients (mean age 14 years) were evaluated. Clinical assessment included patients' symptoms, weight, blood pressure, heart rate, presence of oedema and vascular congestion, before and after dialysis session. Dry weight was assessed based on the above parameters. Fifty-two echocardiographic studies immediately prior and 30-60 min following dialysis were performed. The anteroposterior IVCD was measured 1.5 cm below the diaphragm in the hepatic segment in supine position during normal inspiration and expiration. IVCD was expressed per body surface area. Results. Following haemodialysis mean IVCD (average of expiration and inspiration) decreased from 1.12 ± 0.38 to 0.75 ± 0.26 cm/m 2 (P < 0.0001). Changes in IVCD were significantly correlated with alterations in body weight following dialysis (P < 0.0001). The collapse index (per cent of change in IVCD in expiration vs inspiration) increased significantly after dialysis (P = 0.035). IVCD clearly reflected alterations in fluid status. It did not vary significantly with changes in dry weight in a given patient. Conclusions. Our findings support the applicability of VCD measurement in the estimation of hydration status in paediatric haemodialysis patients. The combination of clinical parameters and measurement of IVCD may enable more accurate evaluation of hydration of children on haemodialysis.

The acute complications of therapeutic cardiac catheterization for congenital heart disease as performed currently in a small unit were reviewed. In recent years, there has been a significant increase in the number of lesions thought amenable to catheter therapy. Only a few reports, however, have addressed the overall incidence of acute complications of therapeutic cardiac catheterization, all representing the experience of centres performing moderate-to-large numbers of procedures. A retrospective review was performed of 425 therapeutic catheter procedures performed at our institution between May 1993 and November 1997. Acute complications were retrieved from the database. This included all adverse events that were clinically recognized at the time of or within 2 weeks after the procedure and which, to the best of the authors' clinical judgement, were related to the catheterization and not part of the natural history of the child's illness. All patients were observed overnight following the procedure, and stayed in hospital if a complication developed. There were 49 acute complications (11.5%), of which 43 (10.1%) were deemed minor and 6 (1.4%) were considered major. The rate was low in patients with valvar pulmonary stenosis, including three neonates (3/45, 6.7%), for those undergoing angioplasty of native co-arctation (1/15, 6.7%) and pulmonary arteries (2/21, 7.4%); and for coil embolization of systemic to pulmonary collateral arteries (1/16, 6.3%). The rate was high in patients with valvar aortic stenosis, including 12 neonates (9/37, 24.3%), and for angioplasty of re-coarctation (4/23, 21.7%). There were more overall complications in neonates (25.6%) than in older patients (10.1%) (p< 0.01). Two patients died (0.5%), but no patient required emergency surgical intervention. In spite of the introduction of many new therapeutic modalities with greater intrinsic risk, and the fact that patients with more complex lesions and who are more acutely ill are being treated, the overall rate of complications remains relatively low. This probably reflects improvements in pericatheterization medical management, in selection of patients, in procedural techniques, and in the experience of operators.

This study retrospectively analyzed the characteristics of infective endocarditis (IE) in children in light of recent advances in pediatric cardiology. We evaluated 25 consecutive patients with IE from 1980 to 1991 at a tertiary Children's Medical Center in Israel. The incidence of IE increased significantly over the second half of the study period, owing mainly to an increase in the number of patients less than 2 years old. Concomitantly, the causative microorganisms changed considerably, with a decrease in viridans streptococci, an increase in staphylococci and other uncommon causes of IE, and increased antibiotic resistance. Children under the age of 2 years, previously considered uncommon IE patients, accounted for 47% of the IE cases during the second half of the study period. The infection often (78%) appeared in children with complex congenital heart diseases, commonly after early palliative or definitive cardiac surgery. Infants who underwent systemic-to-pulmonary shunting, especially with implantation of foreign materials, were at highest risks. IE was more often hospital-acquired (56% versus 6%) and acute (56% versus 44%) in the younger children than in the older ones and often lacked the typical clinical presentation of the disease. In addition, uncommon causative organisms were noted more frequently (67% versus 13%; p = 0.009) in the younger group. The study shows a significant increase in IE in infants and young children with distinctive underlying conditions, clinical presentation, and microbiologic findings, all of which should be considered currently for the diagnosis and treatment of pediatric IE.

The objective of this study was to determine the procedural success rate of balloon angioplasty for branch pulmonary artery stenosis in terms of its clinical impact on the subsequent management of these patients. Most previous studies of balloon angioplasty have concentrated on the initial success rate (50-60%), complications (6-10%), recurrence rate ( approximately 15%), and technical issues. A favorable clinical impact was noted in only 35% of patients. Over a 3-year period (March 1990 to March 1993), 32 patients (17 boys, 15 girls) underwent 34 balloon angioplasty procedures. Their mean age at dilation was 7.6 +/- 4.3 years (range 1.1-19.0 years). Postoperative tetralogy of Fallot and tetralogy of Fallot with pulmonary atresia were the most frequent cardiac lesions (44%). The procedures were "technically" successful in 56% (19 of 34) of balloon dilations on the basis of at least two of the following criteria: an increase of50% of the predilation diameter; an increase of20% in the relative flow to the affected lung by radioisotope study; or a decrease in the systolic right ventricular/aortic pressure ratio from 85-100% to60%. Twelve percent of the patients had transient complications (two deep vein thromboses, one unilateral pulmonary edema, and one pneumothorax). In 17 of 19 (89%) of the patients there was a favorable clinical impact on their subsequent care as based on one of the following criteria: resolution of the stenosis and avoidance of surgical intervention (n = 14); optimization of future surgical procedure (n = 3); reduction in right ventricular pressure to60% of aortic pressure (n = 13). Five patients who had unsuccessful balloon angioplasty and one with initially successful balloon angioplasty later underwent endovascular stent placement, which also favorably influenced their clinical status. The success rate of balloon angioplasty for branch pulmonary artery stenosis, when measured by strict procedural criteria, is accompanied by a favorable clinical impact in more than 50% of patients. Hence this procedure should be the initial therapeutic modality in this setting despite the relatively high transient complication rate. The use of endovascular stents probably increases the favorable clinical impact.

We report on a child with Williams syndrome who died from aneurysm rupture 2 weeks following balloon angioplasty for branch pulmonary artery stenosis.

We compared our current practice of closing small patent ductus arteriosus (PDA) with coils with our previous experience of using double-umbrellas. Twelve patients underwent percutaneous closure of a small PDA with a coil. Selection criteria were a minimal diameter of < or = 2.5 mm and angiographic type A or E. The 12 most recent, non-consecutive patients who had undergone double-umbrella device closure of a PDA and would presently be considered suitable candidates for spring coil occlusion were retrospectively reviewed. The two groups were compared with regard to complications and immediate and midterm results. Eleven of the 12 attempted PDA occlusions using spring coils were successful. The mean follow-up period was 5.8 +/- 4.6 months. Color-Doppler echocardiograms have shown no residual leaks, no turbulence in the descending aorta, and no left pulmonary artery stenosis. All 12 attempted double-umbrella device placements were successful. The mean follow-up period was 16.2 +/- 5.8 months. Color-Doppler echocardiograms have shown trivial residual leaks in four patients and mild turbulent flow in the left pulmonary artery in one patient. There was no significant difference between the two groups in demographic and hemodynamic data. Although the mean follow-up time was significantly longer in the patients who underwent double-umbrella closure, there was significantly more color-Doppler echocardiographic evidence of residual flow (P < 0.03). Small PDA closure with coils is effective, resulting in less residual leaks compared with the double-umbrella device.

Fifty-eight children with syncope were evaluated prospectively to determine the characteristics of syncope in the pediatric age group and the yield of various diagnostic tests. The age at first syncope ranged from 0.5 to 15 years. Twenty-five children presented after a single episode and 33 after multiple episodes. Ten had a history of breath-holding spells. Nineteen had a family history of syncope. A diagnosis was established in 53 patients (91%): vasodepressor (31), cardioinhibitory (13), tussive (3), hyperventilation (2), and mixed syncope (4). In five patients (9%), the cause remained unknown. The diagnosis was established from the history in 45 cases, by a positive oculocardiac reflex in 11, and by the head-up tilt test in four. We conclude that the cause of most cases of pediatric syncope is vasodepressor or cardioinhibitory and can be diagnosed by good history-taking. Costly evaluations are rarely necessary.

Summary In a 29-year-old woman with systemic lupus erythematosus, death was the result of acute myocardial infarction associated with extensive coronary atherosclerosis. The first of two myocardial infarcts was identified at 27 years of age. None of the recognized underlying factors for premature atherosclerosis was identified. An association between lupus and atherosclerosis has previously been reported in a limited number of cases.

The cardiovascular abnormalities were investigated in two unrelated hemizygous males with Fabry disease who had clinical mitral insufficiency. Postmortem examination of their hearts revelaed anatomic, ultrastructural and biochemical abnormalities resulting from defective activity of the lysosomal enzyme, alpha-galactosidase A. The ultrastructural and biochemical studies demonstrated the marked accumulation of the major glycosphingolipd substrate, trihexosyl ceramide, in the lyosomes of all the cardiac tissues examined; the greatest concentrations were found in the mitral valve and elft ventricular myocardium. Intriguingly, digalactosyl ceramide, a glycosphingolipid substrate not detectable in normal lung, vessel or cardiac tissues, was found increased only in the lung and right heart tissues. Morphologic and chemical examination of cardiac and systemic vessels demonstrated accumulation of trihexosyl ceramide in lysosomes of the vascular endothelium. These studies demonstrate that the progressive accumulation of trihexosyl ceramide in the lysosomes of the cardiac structures and vascular system leads to the multiple cardiovascular manfiestation of Fabry disease.

Two siblings with Sandhoff's disease manifested clinical and laboratory evidence of significant cardiomyopathy. Postmortem examination of the hearts of both patients revealed anatomic, biochemical and ultrastructural abnormalities resulting from deficient activities of the enzymes hexosaminidase A and B. A study of the biochemical and ultrastructural changes in the hearts revealed the accumulation of the glycosphingolipid globoside in all cardiac tissues. The necropsy findings indicate that Sandhoff's disease should be considered in the differential diagnosis in infants with cardiomyopathy or mitral insufficiency.

In 20 patients who underwent a modified surgical repair of tetrad of Fallot complete right bundle branch block developed in only 8 (40 percent). Standard and intraoperative conduction studies indicated that in these patients the right bundle branch block was due to injury of the right bundle branch near the ventricular septal defect (proximal right bundle branch block). The modified operative technique is aimed at minimizing the injury to the right ventricle and it includes a significantly shorter than usual ventriculotomy incision and avoidance of the septal (moderator) band during infundibulectomy. Intra- and postoperative hemodynamic studies of these patients revealed that relief of the right ventricular outflow obstruction was optimal.

A developmental complex is described characterized by (1) supravalvular stenosis of the aorta and pulmonary trunk, (2) dysplasia of valves and (3) stenosis of ostia of coronary arteries and branches of the aortic arch. From the four cases described, it is evident that not all of the features of the complex need be present in a given case and, when present, some lesions are not of functional significance. The variations underly the potential for differences in the hemodynamic states of affected subjects.

The plain roentgenographic and angiocardiographic features of 15 patients with Ebstein's anomaly of the tricuspid valve were reviewed. Of note on the plain roentgenogram was the fact that the "posterior bulge" seen on the lateral view was demonstrated to be due to either a markedly enlarged right atrium or the posterior displacement of normal left heart structures. The angiocardiographic features described in the literature were reviewed. Two main features seen were the "sail-like" appearance of the anterior leaflet of the tricuspid valve, which sometimes produces a tumor like picture, and the 2 notches seen when the ventricle contracts. One notch corresponds to the true tricuspid annulus. The other represents the site of attachment of the displaced tricuspid valve. The previously undescribed features were noted: (a) all three leaflets could sometimes be identified; (b) fenestrations in the anterior leaflet were demonstrated in 5cases; and (c) the sinus portion of the ventricle was sometimes filled in a retrograde fashion from the infundibular chamber.

Aortico-left ventricular tunnel (ALVT) is an extremely rare congenital entity in which an abnormal communication between the aorta and the left ventricle bypasses the aortic valve. The condition usually results in gross aortic regurgitation, rapid cardiac decompensation, and death. An additional case of ALVT managed by successful surgical repair is described. The 38 cases reported in the literature are reviewed with emphasis on the clinical and pathological features, diagnosis, natural history, and management. Early operation is recommended to prevent distortion of the aortic valve, dilatation of the left ventricle, and distortion of the aortic anulus.

Ten cases of ventricular septal defect associated with aortic insufficiency are presented. In this syndrome the aortic insufficiency is a complication of the ventricular septal defect. The underlying anatomy is reviewed and a possible explanation for the presence of the aortic insufficiency is suggested.The roentgenographic and cardioangiographic features are stressed. Thoracic aortography may reveal characteristic deformities produced by the involved aortic sinuses and their prolapsed leaflets. Selective right ventriculography may aid in the evaluation of the obstruction of the right ventricular outflow tract.

This report presents an experience with 44 children, including 9 less than 5 years of age, who were operated upon for replacement of a cardiac valve. Our early operative mortality was 60%, whereas in the past 4 years it has been only 30%. We have operated only on significantly symptomatic children for whom no other therapy was available. From our experience, we cannot judge the necessity of anticoagulants. No patients have required a second operation because the valve has been outgrown, although several patients who underwent mitral valve replacement have had postoperative gradients across the prosthesis.

Famil ial clustering of ascaris and trichuris carriers was the outstanding finding in this study. In ex tens ive studies of he lmin ths in the 1930s, Cor t and Otto 4, 5 noted that the impor tan t uni t in ascaris infections was the family. A t present several effective drugs o f low toxicity are available for t r ea tment o f ascariasis, and it is felt that asymptomat ic carriage warrants t reatment . 6 Our "red i scovery" of the familial aggregation of ascaris infections therefore has significant implications for communi ty control. The siblings of a child with ascaris are likely to have the parasite, and they should be examined for it. Barring this, we would r e c o m m e n d that the sibling be treated for ascaris at the same t ime as the infected child.

The treatment of atherosclerosis in adulthood does not get to the root of the problem, which is obviously prevention. The latter must of course start at an early age. In order to do this, we must understand the etiology and pathogenesis of atherosclerosis.

Right aortic arch with left descending aorta and a retroesophageal aortic segment is an uncommon congenital vascular malformation which may cause symptoms of compression from a vascular “ring.” The presence of this malformation may be suspected by the findings on barium swallow and the diagnosis confirmed by angiocardiography. Surgical intervention may be necessary in patients who have severe symptoms.

The development of optical fibers capable of transmitting laser energy has encouraged the experimental use of laser irradiation for the treatment of acquired cardiovascular disorders. One of the key questions is which combination of laser source, energy parameters, and transmitting fiberoptic would be best suited for intravascular use. In most experiments argon, neodymium-YAG, and excimer lasers, coupled to suitable optical fibers, have been used. We now describe the use of a carbon dioxide fiberoptic laser catheter for the creation of an atrial septal defect. Silver halide infrared transmitting fibers were inserted into standard 6 French cardiovascular catheters. This laser catheter system, capable of transmitting several watts of pulsed CO2 laser energy, was initially used to create atrial septal defects in isolated dog hearts to determine the best energy parameters. Atrial septostomy was later performed successfully in four of five anesthetized dogs. The thermal damage extended 50 to 60 micron beyond the "holes" created by the laser irradiation in the interatrial septum. Thus, pulsed CO2 laser irradiation, delivered through optical fibers, can create an atrial septal defect.

In an infant with patent ductus arteriosus and pulmonary hypertension, elevated pulmonary vascular resistance persisted following ligation of the ductus. Histologic examination of a pulmonary biopsy at two years of age and tissue obtained at autopsy at three years of age showed obstructive pulmonary vascular disease. The question as to whether the organic vascular lesions are secondary to the effects of the patent ductus or part of pulmonary hypertension cannot be resolved. The age of the patient favors a primary etiology.

Early vascular complications and late sequelae are reported in relation to 195 left heart catheterizations, 125 performed by brachial arteriotomy (BA), and 70 by percutaneous femoral artery puncture (FAP) with the Seldinger technique. Vascular complications occurred in 25 patients, 11 requiring surgical intervention, which was successful in restoring normal circulation in all. The rate of complications was higher after FAP in children 6 years old or less, while after BA the rate was significantly higher in patients of 2 years or older. In patients under 2 years, brachial artery suture repair resulted in more complications than ligation, but the cases were not randomized. Late follow-up of 101 catheterizations (35 FAP, and 66 BA, of which 38 were ligated and 28 sutured) included clinical evaluation, Doppler pressure measurements, and a comparison of the length and bone development of the catheterized and non-catheterized limbs. All these have indicated an absence of any serious late sequelae in this group 6 months to 9 years (average 4 years) after catheterization.

The anatomic and ECG features of 23 patients with polysplenia and 17 patients with asplenia were reviewed. Patients with polysplenia tended to have a superiorly oriented P wave axis. In asplenia the P wave axis was inferiorly directed, but not in the normal location. We were unable to correlate the abnormal P wave axis with specific anatomic features of the atria.

A 6-day-old neonate with tricuspid atresia and transposition of the great vessels developed acute bacterial endocarditis and the "scalded skin syndrome." A nonphage typable Staphylococcus aureus was recovered from multiple sites prior to death, and staphylococci were also cultured from vegetations on the endocardial surface of the heart at necropsy. This case represents one of the few documented cases of bacterial endocarditis in the neonatal period and is one of the first associated with congenital heart disease. It is also the first recorded case of the scalded skin syndrome associated with bacterial endocarditis.

A case of congenital absence of the pulmonary valve associated with tricuspid atresia and intact ventricular septum is presented. In this case, the presence of a patent ductus arteriosus and absent pulmonary valve allowed the right ventricle to receive blood from the pulmonary artery. The right ventricle, however, represented a blind pouchlike structure which was not an essential part of the circulation.