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This paper proposes a customized and versatile optical setup to evaluate the optical performance of different commercially available intraocular lenses (IOLs). The setup was used to measure the wavefront error induced by different IOL models, verifying and analyzing the magnitude of low- and high-order aberrations induced by currently available IOLs. Independent analyses included non-toric spherical and aspheric IOLs from three different manufacturers. Three different dioptric powers were tested: 15, 20, and 25 D from each model. Dioptric power, induced cylinder, and spherical aberration were measured in each tested lens. All lenses had dioptric power within the tolerated margin of error. Induced cylinder was also within the current standards and clinically irrelevant. Spherical aberration varied according to the analyzed dioptric power and to the IOL model. We reported on the importance of the plane where the spherical aberration is reported, IOL or cornea. All analyzed IOLs were within current standards for the dioptric power and induced cylinder. Spherical IOLs had higher spherical aberration measured at the IOL plane.

Purpose: To analyze the optical performance of different implant strategies in simulated nanophthalmic eyes., Methods: An optical design software was used. Analysis included eye models that required 30.00, 45.00, and 60.00 diopters (D) intraocular lenses (IOLs) to achieve emmetropia. Spherical and aspheric IOLs were designed. They were tested either with a single implant setting S or by splitting the power into two lenses. Setting P1 had an even split of the power between the lenses and setting P2 had an uneven power split with one-third of the power in the anterior lens and two-thirds in the posterior IOL. The area under the modulation transfer function (MTF) curve was calculated and spherical aberration was recorded in each setting., Results: Setting S had the worst optical performance in the spherical group and the best performance in the aspheric group. A statistically significant difference was found between setting S and the piggyback options (settings P1 and P2) in all analyzed variables for the spherical and aspheric groups for the 45.00 and 60.00 D IOL requirement. No statistically significant difference was found between the piggyback settings., Conclusions: Single aspheric IOLs had better optical performance than piggybacking lower-power aspheric IOLs. In the spherical lenses group, the results were the opposite, with the piggyback options having higher optical quality than the single IOL. MTF shows that single aspheric lenses provide the highest contrast sensitivity among all of the analyzed settings., (Copyright 2016, SLACK Incorporated.)

The objective of this paper was to describe the outcomes in patients submitted to arterial switch operation and to analyze the predictors of in-hospital mortality and further need of re-operation at a single-center institution. Between September 1995 and January 2014, 128 consecutive arterial switch operations were performed. Surgical mortality during this period was analyzed retrospectively, and a follow-up analysis of the survivors was conducted. Surgical era, cardiopulmonary bypass time (p = 0.001), and diagnosis category (p = 0.025) influenced in-hospital mortality. The estimated overall survival for the 91 hospital survivors was 96.8, 96.4, and 96.2 % at 5, 10, and 15 years, respectively. The median follow-up time was 67 months (range 0.71-222 months). Three patients (5 %) presented severe aortic regurgitation. Right ventricle outflow tract systolic gradient by echocardiography was above 60 mmHg in 2 %. Late re-interventions occurred in 12 (13 %) patients with mean time of 64 ± 34 months after the initial procedure. Actuarial freedom from re-interventions at 5, 10, and 15 years was 96.4, 69.7, and 61.9 %, respectively. Arterial switch operation remains the procedure of choice in patients with transposition of great arteries. It can be performed even in middle-volume institutions, leading to the same middle- and long-term outcomes of high-volume institutions. Early high mortality rate may occur due not only to learning curve, but also to cardiopulmonary bypass time and ventricular septal defect closure.

During the last decades, advances in diagnosis and treatment of congenital heart disease have allowed many individuals to reach adulthood. Due mainly to the great diagnostic diversity and to the co-morbidities usually present in this age group, these patients demand assistance in a multidisciplinary facility if an adequate attention is aimed. In this paper we reviewed, based in the international literature and also on the authors' experience, the structural conditions that should be available for these patients. We highlighted aspects like the facility characteristics, the criteria usually adopted for patient transfer from the paediatric setting, the composition of the medical and para- medical staff taking into account the specific problems, and also the model of outpatient and in-hospital assistance. We also emphasized the importance of patient data storage, the fundamental necessity of institutional support and also the compromise to offer professional training. The crucial relevance of clinical research is also approached, particularly the development of multicenter studies as an appropriate methodology for this heterogeneous patient population.

Objective: Autonomic dysfunction is an important mechanism that could explain many symptoms observed in fibromyalgia (FM). Exercise is an effective treatment, with benefits potentially mediated through changes in autonomic modulation. Strengthening is one of the less studied exercises in FM, and the acute and chronic effects of strengthening on the autonomic system remain unknown. The objective of this study was to assess the chronic effects of strengthening exercises (STRE) on autonomic modulation, pain perception and the quality of life (QOL) of FM patients., Methods: Eighty sedentary women with FM (ACR 1990) were randomly selected to participate in STRE or flexibility (FLEX) exercises in a blinded controlled trial. The intensity of STRE was set at 45% of the estimated load of 1 Repetition Maximum (RM) in 12 different exercises. Outcomes were Visual Analog Scale (VAS) for pain, Heart Rate Variability (HRV) analysis, treadmill test, the sit and reach test (Wells and Dillon's Bench), maximal repetitions test and handgrip dynamometry; and quality of life by the Fibromyalgia Impact Questionnaire (FIQ), the Beck and Idate Trait-State Inventory (IDATE), a short-form health survey (SF-36)., Results: The STRE group was more effective to strength gain for all muscles and pain control after 4 and 16 weeks (p<0.05). The FLEX group showed higher improvements in anxiety (p<0.05). Both groups showed improvements in the QOL, and there was no significant difference observed between the groups. There was no change in the HRV of the STRE and FLEX groups., Conclusions: Strengthening exercises show greater and more rapid improvements in pain and strength than flexibility exercises. Despite the benefits in fitness, pain, depression, anxiety and quality of life, no effect was observed on the autonomic modulation in both groups. This observation suggests that changes in autonomic modulation are not a target tobe clinically achieved in fibromyalgia., Trial Registration: ClinicalTrials.gov NCT02004405.

Objective: To report the institution experience with the surgical treatment of adults with congenital heart disease due to the increasing number of these patients and the need for a better discussion of the subject., Methods: Retrospective analysis describing demographic data, risk factors and results., Results: 191 patients between 16 and 74 years old were operated on. Primary correction was done in 171 cases, 93 (55%) for atrial septal defect repair. Among 20 (12%) reoperations, pulmonary valve replacement was done in six cases. The mean intensive care and hospital stay were 2.7 and 8.5 days respectively, significantly greater for the reoperated cases (P=0.001). The mean bypass and clamping times were 68.6 and 44.7 minutes respectively, greater for the reoperated cases (P<0.0001 and P=0.0003 respectively). Hospital mortality was 4.2% and male sex, functional class III-IV and older age at operation were predictive risk factors. Significant complications were more frequent in the reoperated cases (P<0.003), mainly atrial flutter and fibrillation. Among 183 patients discharged, 149 (82%) are being followed and atrial flutter and fibrillation are common. The mean functional class value improved significantly after operation (1.66 to 1.11; P<0.0001). The estimated survival was 96.2% in six years., Conclusion: Heart surgery in adults with congenital heart disease can be accomplished with low mortality and functional class improvement. Immediate and late complications are frequent. Multicenter studies are important to better characterize this patient population in the country.

Purpose: To present an alternative methodology to characterise intraocular lenses (IOLs) using an optical setup with a Hartmann-Shack wavefront sensor., Methods: The methodology was inspired by the ANSI Z80.30 Standard, which addresses IOL optical properties and test methods, and aims to simplify the procedure to allow complete IOL characterisation with a reduced number of measurement steps., Results: Seven spherical and three toric IOLs were characterised with the alternative methodology and by commercial equipment. Three spherical IOLs measured with the alternative methodology obtained dioptric spherical errors larger than the tolerance established by the ANSI Z80.30 Standard when compared to the IOLs theoretical designs, probably due to manufacturing deviations. All cylindrical dioptric power measurements were within the Standard tolerance. The longitudinal spherical aberration of the IOLs were measured and 2-dimensional Modulation Transfer Functions (MTF) were calculated in order to complementarily assess manufacturing and design quality., Conclusions: The alternative methodology allows complete IOL characterisation with a reduced number of steps compared to those required by the Standard ANSI Z80.30, achieving satisfactory results in measurements of IOL dioptric power, spherical aberration, cylindrical power and MTF. Although MTF measurements were not performed with a model eye, they may contain additional relevant information regarding the design of ophthalmic lenses., (© 2013 The Authors Ophthalmic & Physiological Optics © 2013 The College of Optometrists.)

This study was carried out to investigate the effects of chemical lesions of dorsal periaqueductal gray (DPAG) on resting arterial pressure (AP) and heart rate (HR) as well as on cardiac baroreflex of conscious normotensive rats. Lesions were performed by bilateral microinjections of 150 mM NMDA into the DPAG (DPAG-lesion group). Controls were similarly injected with 165 mM NaCl (DPAG-sham group). Animals with chronic lesions confined only to the superior colliculus (SC-lesion group) were also used as controls of DPAG-lesion. Cardiovascular parameters were recorded 1 or 7 days after the microinjections of NMDA in acute and chronic groups, respectively. Cardiac baroreflex was assessed by measuring the HR responses to the intravenous injection of phenylephrine or sodium nitroprusside. Baroreflex was estimated by sigmoidal curve fitting of HR responses. An increased baroreflex gain was observed in chronic DPAG-lesion rats compared to both DPAG-sham (p < 0.01) and SC-lesion (p < 0.05) chronic groups. The chronic DPAG-lesion group showed also an elevation of both the tachycardia (p < 0.05) and bradycardia (p < 0.01) plateaus compared to chronic DPAG-sham rats, while the SC-lesion group showed an elevation of the bradycardia plateau only (p < 0.01). Similar results on baroreflex function were observed following acute lesion of the DPAG, i.e. an increase in baroreflex gain (p < 0.01) and the elevation of both tachycardia (p < 0.05) and bradycardia plateaus (p < 0.01) compared to the acute DPAG-sham group. Resting AP and HR did not differ among the chronic groups. In contrast, the acute lesion of the DPAG produced a reduction in AP (p < 0.01) accompanied by an increase in HR (p < 0.01). The present data suggest that the DPAG is involved in the tonic and reflex control of AP and HR in conscious rats. In addition, the SC seems to contribute to the baroreflex cardioinhibition.

We report the case of a neonate admitted to the hospital in the 4th day of life in severe heart failure due to aortic and mitral regurgitation with a largely dilated aortic root. The associated skeletal features involving the superior and inferior limbs as well as the thorax, and joint hypermobility, allowed the clinical diagnosis of Marfan syndrome. Despite favorable initial response to medical therapy, sudden deterioration led to death two weeks after birth. Typical necroscopic findings were confirmed and the case is considered the most severe clinical manifestation possible to be found in this syndrome.

Aiming to document the incidence and type of associated congenital heart disease, 20 children affected with the congenital rubella syndrome have been evaluated during a 5 year period, starting 1988. Congenital heart disease was detected in 45% of the cases. Persistent ductus arteriosus was the most frequent finding, followed by ventricular and atrial septal defects. Hearing disturbances occurred in 83% of the 12 cases studied. Ophthalmic lesions appeared in 69% of the 13 cases analysed, congenital cataracts being the most frequent one. An association of 66% was found between ophthalmic and heart lesions. Regarding the nutritional index, 70% of the cases were situated between the 25th and 50th percentile. The relevant incidence of congenital defects and the documentation of an important number of cases in a short period of time point out to a significant prevalence of the syndrome with imperative need improve the vaccination programs.

A case of newborn intrapericardial teratoma is reported. The clinical, echocardiographic, tomographic and histologic features are described, and also, the therapeutic options. The newborn was submitted to surgical excision of the intrapericardial tumor and has a clinical follow-up greater than four years.

Two cases of congenital trabecular hypoplasia of the right ventricle are reported. In the first, the neonatal diagnosis was missed and the child did well until the 13th month of life when a modified Blalock-Taussig shunt was done because of increasing cyanosis. Outcome was good until the 4th year of life when symptomatic atrioventricular block was detected in an emergency situation. A bidirectional Glenn anastomosis and pacemaker implantation were successfully carried out after clinical establization and the child is doing well up to now. The second case presents the disease with its worst features: severe cyanosis and acidosis in the first day of life. A modified Blalock-Taussig shunt was performed and death occurred soon after the operation.

Two thousand consecutive children have been evaluated for suspected heart disease in a 27-month period. The main reasons for referral were: murmur (70%), precordial pain (9.5%), suspicion of arrhythmia (8.5%) and breathless (5%). Five hundred and six (25%) cases did not complete the investigation and the results were not computed. A final diagnosis was obtained based on the reason for referral and the main conclusions were: 1) a high incidence of normality was found: murmur (83%), pain (98%), arrhythmia (97%) and breathless (94%); 2) heart disease is unlikely when other referral reasons were analyzed; 3) 14% of the children were considered abnormal and the necessity of therapeutic procedures was 0.8%. A pediatric cardiology outpatient clinic in a public setting seems to be justifiable in the region, due to the high current demand.

Purpose: To determine the benefits of noninvasive tests in the characterization of heart disease in children referred due to heart murmur., Methods: Two hundred and thirty three consecutive children were fully examined with the aid of the electrocardiograma (ECG), chest X-ray and echocardiogram (ECHO). The patients were divided in 2 groups according to the initial diagnosis of innocent murmur (n = 23) and pathological murmur (n = 210). A comparison was made between the initial and final diagnosis after the noninvasive tests. Statistical analysis was employed through the qui-square test., Results: The initial diagnosis did not change after ECG. The chest X-ray induced the diagnosis of idiopathic dilatation of the main pulmonary artery in one case and of cardiomyopathy in 8 cases, confirmed in only one. After ECHO, the initial diagnosis of innocent murmur was maintained in 70% of the cases, while 30% had some form of heart disease. In 80% of the pathologic murmur group, the diagnosis did not change after ECHO, while 20% were considered as having an innocent murmur. In the 2 groups together, the concordance index between initial and final diagnosis after ECHO reached 90%. Statistically, a non-significant difference was found between the 2 groups., Conclusion: Careful clinical examination is mandatory in the initial evaluation of children with heart murmur. ECG and chest X-ray does not change the initial clinical diagnosis. After ECHO, the diagnosis changing was low (10%), as long as clinical mistakes are eliminated. The initial diagnosis of innocent murmur excludes the necessity of noninvasive tests with a small possibility of error. EKG, chest X-ray and ECHO should be reserved to cases with obvious or persistent suspicious heart disease after clinical examination.

Eighty-six patients with Down Syndrome were studied with the main purpose of quantifying the incidence of congenital heart defects and the risk of occurrence according to the mothers age. Thirty-eight patients had the cariotypes determined, 35 of them having trissomy of chromosome 21 and translocation in 3 cases. Congenital heart disease was found in 44 (51%) of the patients, the most common one being ventricular septal defect. An important incidence of Fallot's tetralogy was also found (20%). These 86 children were submitted to 41 surgical procedures, most of them on the cardiovascular system. The maternal mean age was 33 -/+ 8.6 years and the estimated risk of Down Syndrome was 1/590, a lower value than the one reported in other studies.

A case of 3 month-old-boy with Down's syndrome and heart failure due to a localized arteriovenous fistula of the cranial vessels is reported. Clinical diagnosis was made after cranial bruit detection based on a retroauricular thrill incidentally felt by the mother. The diagnosis was confirmed by digital subtraction angiography and a good result was achieved by means of embolization and surgical ligation of the fistula. Three years after the operation the patient is asymptomatic, on no medication and with normal magnetic resonance image of the cranial vessels.

Purpose: To determine the frequency and main features of subsequent cardiovascular surgery in patients operated on for coarctation of the thoracic aorta., Methods: One hundred and five patients operated on for coarctation of the aorta with a mean follow-up period of 14 years had their notes analysed. The patients were divided in 4 groups according to age at correction of the coarctation. The incidence of recoarctation repair and other cardiovascular operations were noted., Results: Surgical morbidity was important: 33%. Recoarctation occurred in 14% of the cases, mainly in those who had the coarctation resected during the first year of life. Operation for other cardiovascular defects was necessary in 24.5% of the cases. Among these, correction of left-to-right shunts was done in 50% of the patients who had the coarctation resected in the first year of life. Relief of aortic stenosis was the most frequent procedure (73%), tends to be more frequent the other is the patient at coarctation repair and more than one procedure may be necessary in some cases., Conclusion: Reoperation is frequent in the long term of patients operated on for coarctation of the aorta. The elective coarctation should be repaired after the first year of life hoping to avoid recoarctation. Routine follow-up is advisable for all patients aiming to detect residual left-to-right shunts and left ventricular outflow tract obstruction. Family counseling regarding prognosis after coarctation resection is recommended.

The case of a symptomatic 16-year-old girl, referred with the diagnosis of coarctation of the aorta is presented. Aortography showed a severe coarctation and a huge aneurysm distal to the coarctation. Successful operation was performed with resection of the coarctation and aneurysm and interposition of a tube of bovine pericardium for the anastomosis. Two months after the operation the blood pressure was normal and a 10 mm/Hg gradient was detected by doppler-echocardiography.

The case of an asymptomatic 6-month-old boy, first seen due to a right arm edema is described. A continuous murmur heard at the right infraclavicular area, cardiomegaly plus increased flow to the lungs on the chest X-ray were associated with an isolated right subclavian artery-to-vein fistula diagnosed at angiography. Successful ligation of the fistula was done at operation with normalization of the arm circumference and heart size on the chest X-ray at 4 years follow up.

Traumatic rupture of the interventricular septum is a rare condition usually presenting in infancy and childhood. This report describes two cases: a 3-year-old boy operated on soon after the diagnosis was made, and a 15-year-old girl operated on 12 years following the causative accident. Both patients had the diagnosis confirmed by cardiac catheterization. The typical appearance of a traumatic ventricular septal defect (VSD) was seen during surgical repair that was successfully carried out in both patients. The protracted clinical course of the second patient, eventually requiring corrective intervention, contributes to the understanding of natural history of traumatic VSD.

A patient with severe hypertrophic cardiomyopathy and heart failure at six weeks of age is reported. It was believed that myocardial infarction had occurred based on the striking ECG changes and the histologic appearance of the myocardium at necropsy.

The case of a patient with varicella skin lesions presenting with heart failure at 4 years of age is described. A transitory atrioventricular conduction disturbance due to myocardial impairment was documented during hospital stay. Despite the clinical improvement with conventional therapy, the patient died from respiratory failure due to a lung infection but with an almost normal electrocardiogram. Necropsy showed myocardial lesions compatible with acute myocarditis. A possible viral specific affinity for the conduction system of the heart is suggested and judged to be a potential cause of death in this disease. Since the actual incidence of varicella myocarditis associated to an atrioventricular conduction disturbance is unknown it seems prudent that every patient with varicella infection should have a cardiac examination during the acute phase of the disease. As the diagnosis of subclinical myocarditis depends essentially on an electrocardiogram, we believe this method should be employed routinely during the acute phase in order to assess myocardial involvement in this disease, which may be a cause of sudden death.

The two techniques usually employed for the surgical treatment of tricuspid valve endocarditis are valve replacement or simple valve excision without valve replacement. A third more conservative procedure consists of resection of the vegetation ("vegetectomy") and leaflet repair, if necessary. Valve excision without valve replacement is described in adult patients especially in addicts of intravenous drugs, with normal ventricular function. Two cases of tricuspid valve endocarditis in children with interventricular communication successfully treated by "vegetectomy" and valve excision without valve replacement are reported. In the first case, surgery was performed during acute infection. Contrarily in the other case there was evidence that cure had been obtained, but still the prosthesis was not implanted due to the excellent hemodynamic status at the moment, and to abbreviate bypass time due to the preoperative conditions of the child. The uncommon approach to these two cases of tricuspid valve endocarditis in childhood motivated this presentation.

Coronary recanalization with thrombolytic agents is a new therapeutic approach to the treatment of acute myocardial infarction that can be beneficial even to patients in cardiogenic shock. Although few cases have been reported in the literature, treatment of acute occlusion of the left main coronary artery (LMCA) has been made possible by myocardial reperfusion. This communication concerns a patient with acute LMCA occlusion who was successfully treated by thrombolytic therapy with streptokinase followed by revascularization of the myocardium seventy-two hours after reperfusion was achieved.

A 57-year-old woman developed severe substernal chest pain radiating to the left arm accompanied by pallor and marked diaphoresis. These symptoms appeared at rest, lasted 45 minutes, and terminated spontaneously. The patient had been treated for mild hypertension during the last 6 months. An ECG tracing obtained at the beginning of treatment was unremarkable. However, an ECG tracing recorded shortly after the end of the symptoms showed T-wave inversion in all anterior leads. Coronary arteriography was then performed and showed no fixed obstructive coronary artery disease. Nonetheless, a lengthened and constricted myocardial bridging of both the left anterior descending coronary artery and its major diagonal branch was detected. Also, the left anterior descending coronary artery was observed to be very short, terminating before the cardiac apex. The left ventricle was hypertrophied. The patient was treated with a beta-blocking agent which eliminated all symptoms. An ECG tracing obtained about three months after the onset of the clinical picture was normal. Our findings suggest that marked myocardial ischemia at rest does occur in patients having myocardial bridges under special circumstances, such as lengthened and constricted myocardial bridging of a short coronary artery which supplies a hypertrophied ventricle. This anomaly should be taken into account as a possible cause of a threatened myocardial infarction, which may be successfully treated with a beta-blocking agent.