Nonrestrictive Ventricular Septal Defect Research Articles

THE POSSIBILITY OF PREDICTING LEFT VENTRICULAR DYSFUNCTION IN THE EARLY POSTOPERATIVE PERIOD IN CHILDREN WITH VENTRICULAR SEPTAL DEFECT

Highlights The article reflects data of an original study on the diagnosis of early postoperative dysfunction in children under 1 year of age after correction of a ventricular septal defect. The relationship between troponin I and conventional echocardiographic parameters, left ventricular longitudinal strain (S, % LV), and parameters reflecting central hemodynamics was assessed. The predictive ability of S, % LV in predicting a decrease in cardiac index after surgery was revealed. AbstractAim. To evaluate the possibility of predicting left ventricular (LV) dysfunction in the early postoperative period in children with ventricular septal defect (VSD).Methods. The study included 204 children under 1 year of age with non-restrictive perimembranous VSD. All parameters were evaluated before surgery, 6 hours, 24 hours and 10 days after surgery. 125 healthy children were enrolled as the control group.Results. 6 hours after surgery, there was a statistically significant decrease in LV ejection fraction (EF) (Me 55 [K3 60–K1 45] %, p = 0.0001), stroke index (SI) of LV outflow tract (LVOT) (12, 07 [16.38–8.24] mL/m2, p = 0.0001), and cardiac index (CI) (1.52 [2.08–1.07] L/min/m2, p = 0.0001). Arterial elasticity (AE) increased (21.4 [32.4–15.79] mm Hg/mL, p = 0.002), indicating a significant increase in afterload. Arterial compliance (AC) decreased (0.058 [0.081-0.038] mL/mm Hg, p = 0.0001), whereas ventricular-arterial coupling (VAC) value significantly increased (2 [2.54–1.67], p = 0.009). There was a dependence between LVOT SI and age (Rs = 0.45, p = 0.03), a moderate relationship between LVOT SI and VAC (Rs = –0.59, p = 0.001), a pronounced relationship between LVOT SI and AC (Rs = 0, 93, p = 0.02), pronounced relationship between LVOT SI and AE (Rs = –0.94, p = 0.03), moderate correlation between VAC and SI (Rs = –0.44, p = 0.04), weak relationship between VAC and patient age (Rs = –0.28, p = 0.03). Longitudinal deformity before surgery (b = 0.028; F = 3.9; p = 0.006) and VSD size (b = -0.08; F = 3.4; p = 0.029) were significantly associated with LV SI dynamics 6 hours after surgery. Dependence equation was as follows: 2.8 – 0.08 * VSD + 0.028 * S. A predictive ability to reduce CI 6 hours after surgery was revealed: longitudinal strain (S), % with a threshold “–” 14.3% (sensitivity 96.2%, specificity 81.6%, AUC 0.92, p<0, 00001) and VSD diameter with a threshold of 8 mm (sensitivity 75%, specificity 52%, AUC 0.69, p<0.0001).Conclusion. In the early postoperative period, there is a decrease in systolic and LV pump function. High AE rates are associated with a decrease in AC and an increase in VAC. Preoperative predictors of SI decrease are as follows: S, %> “–” 14.3 and VSD size > 8 mm.

Performance of remote health education via WeChat to improve the pre-operative nutritional status of infants with non-restrictive ventricular septal defects: A prospective randomised controlled study.

This study aimed to explore the effect of performing remote health education via WeChat to improve the pre-operative nutritional status of non-restrictive ventricular septal defects (VSD). A prospective randomised controlled study was conducted in a provincial maternity and child hospital in China. Participants were randomised regarding education to the intervention group (WeChat) and the control group (leaflets). The nutritional status and complications of the patients were compared after intervening for 1month. Nutrient status comparison at 1month after intervention showed that the body weight, head circumference, haemoglobin, albumin and pre-albumin of the WeChat group were significantly higher than those of the leaflet group (P < 0.05). The STRONGkids score of the WeChat group was significantly lower than that of the leaflet group (P < 0.05). The incidence of feeding intolerance and respiratory tract infection in the WeChat group was significantly lower than that found in the leaflet group (P < 0.05). There was no significant difference in the incidence of liver insufficiency and jaundice between the two groups (P > 0.05). Providing pre-operative feeding and care guidance for parents of infants with non-restrictive VSD, via remote health education through WeChat, can effectively improve nutritional status and reduce the risk of malnutrition and feeding complications.

Prenatal diagnosis of tetralogy of Fallot with a restrictive ventricular septal defect

Objective Tetralogy of Fallot (TOF) usually presents with a large non-restrictive ventricular septal defect (VSD). Case(s) We hereby report a rare case of prenatal diagnosis of TOF with a restrictive VSD secondary to redundant tricuspid valve tissue. Partial occlusion of the VSD resulted in prenatal progression to myocardial hypertrophy and postnatally to right heart failure with difficult management. Conclusion Prenatal signs of cardiac dysfunction in TOF should prompt a detailed exam of the VSD. Parents and neonatologists must be advised and delivery must be guaranteed in a tertiary center.

Benefits of perioperative sildenafil therapy in children with a ventricular septal defect with pulmonary artery hypertension on early surgical outcomes.

Pulmonary hypertension is a common association in children with nonrestrictive ventricular septal defect. It increases perioperative mortality and morbidity. Oral sildenafil is an effective pulmonary vasodilator. In this study, we assessed effects of perioperative oral sildenafil therapy on pulmonary artery pressure and early surgical outcomes. This was a single centre, prospective randomized control study. Thirty children with nonrestrictive ventricular septal defects with pulmonary hypertension were divided into two groups. In the sildenafil group (n = 15, mean age 23.3 months), oral sildenafil was administered two weeks before surgery. In the control group (n = 15, mean age 36 months), preoperative sildenafil was not given. Sildenafil was continued postoperatively in both groups, provided the postoperative pulmonary artery pressure was over 50% of systemic pressure. There was no perioperative mortality, pulmonary hypertensive crisis and there were no intolerable side effects related to sildenafil in either group. Mean pulmonary artery pressure showed a reduction in both groups. Sildenafil group showed statistically significant improvement in duration of cardiopulmonary bypass (100.27 ± 21.09 minvs. 125.40 ± 26.83 min, p = .008), mechanical ventilation requirement (22.79 ± 17.13 hvs. 30.53 ± 13.05 h; p = .04), epinephrine requirement (22% patients vs. 48% patients; p = .03) and hospital stay (6.13 ± 1.40 days vs. 7.53 ± 1.92 days; p = .05). Oral Sildenafil therapy is an inexpensive and well-tolerated method for reducing pulmonary hypertension secondary to non-restrictive ventricular septal defect. It has noteworthy advantages regarding early surgical outcomes like reduced cardiopulmonary bypass time, improved mechanical ventilation time, lower inotrope requirement and shorter hospital stay if used preoperatively in select patient population.

The Effect of Perioperative Administration of Treprostinil in Infants with Non-restrictive Ventricular Septal Defect and Severe Pulmonary Arterial Hypertension.

This study investigated the efficacy and safety of intravenous treprostinil during the perioperative period in infants with non-restrictive ventricular septal defect (VSD) and severe pulmonary arterial hypertension (PAH) undergoing surgical VSD repair. This was a retrospective study. There were 79 infants with non-restrictive VSD and severe PAH receiving surgical treatment from January to December 2019 in our cardiac center. The patients were divided into the treprostinil group and control group according to whether intravenous treprostinil was used during the perioperative period. There were no significant differences in the preoperative characteristics, including age, sex, weight, ventricular size, or preoperative pulmonary artery pressure, between the two groups. Although the pulmonary artery pressure in both groups was significantly lower postoperatively than preoperatively, the postoperative pulmonary artery systolic pressure was significantly lower in group T than in group C. The postoperative mechanical ventilation time, ICU stay, and hospital stay in group T were shorter than those in group C. Treprostinil can be used effectively and safely to reduce the perioperative pulmonary artery pressure in infants with non-restrictive VSD and severe PAH undergoing surgical VSD repair.

P1445 DORv and window: a case of an uncorrected double outlet right ventricle with d-transposition of great arteries with concomitant aortopulmonary window in a 31-year-old female

Abstract Funding Acknowledgements none Double outlet right ventricle (DORV) is a rare type of ventriculo-arterial connection occurring in about 1-3% of congenital heart defects. Moreover, transposition of the great arteries, wherein the aorta is anterior and to the right of the pulmonary artery (D-TGA), accounts for only 26% of cases of DORV. An aortopulmonary window, on the other hand, represents approximately only 0.2% of all congenital cardiac lesions. A case of DORV with D-TGA and pulmonary stenosis with a concomitant aortopulmonary window has never been reported, particularly in an adult in the third decade of life and without any history of surgical repair. We present the case of a 31-year-old female who had sought consult for left-sided weakness. On physical examination, a grade IV/VI systolic murmur was heard at the left anterior chest and cyanosis of the digits of the upper and lower extremities were noted. Transthoracic echocardiogram revealed a non-restrictive ventricular septal defect (VSD) and segmental approach showed atrial and visceral situs solitus, levocardia, and atrioventricular concordance. However, there was ventriculoarterial discordance with fusiform aneurysmal dilatation of the aorta, which was noted to be at the right of and anterior to the pulmonary artery. Both great vessels arose from the right ventricle. Other echocardiographic findings included dilated right atrium, dilated and hypertrophied right ventricle with volume and pressure overload, global left ventricular hypokinesia with mild systolic dysfunction, and moderate to severe aortic regurgitation. The above complex congenital anomalies were confirmed via transesophageal echocardiogram and cardiac magnetic resonance imaging (MRI). Furthermore, pulmonary valve stenosis and aortopulmonary window, that was 3.5 centimeters in length located at the level of the proximal ascending aorta and main pulmonary artery, were seen on the MRI. Various cardiac imaging modalities aided in cinching the diagnosis for this very rare complex congenital case in an adult reaching the third decade of life. Palliative care was deemed best for this patient due to the severity of her cardiac condition. Abstract P1445 Figure 1.

Prenatal Identification of Restrictive and Non-restrictive Ventricular Septal Defects Based on End-Systolic Flow Patterns in the Fetal Aortic Isthmus.

Fetuses with large ventricular septal defects (VSDs) must be closely monitored during the perinatal period. Intrauterine assessment of size of septal defects with bidimensional echocardiography are unreliable. The objective of the present study is to document the contribution of flow pattern analysis in the fetal aortic isthmus for prenatal identification of large non-restrictive VSDs requiring immediate postnatal attention. We conducted a cross-sectional retrospective study of Doppler recordings from patients referred to our Fetal Cardiology Unit and diagnosed with one or multiple VSDs from January 2006 to November 2015. Three groups were defined: (1) large non-restrictive VSDs (n = 11) with postnatal cardiac failure (2) small restrictive VSDs (n = 28) asymptomatic, and (3) absence of cardiac abnormality (n = 66). The Isthmic end-Systolic Index (ISI) was computed to quantify aortic isthmus flow and compared between the three groups. Fetuses with restrictive VSD and control group showed similar ISI: stable at 0.20 ± 0.01 up to 27weeks of gestation and dropping later due to the fall of end-systolic velocities in the aortic isthmus. By 35weeks, a brief end-systolic retrograde flow was present, associated with a further fall of ISI down to -0.26 ± 0.05. In contrast, ISI of fetuses with large non-restrictive VSDs were unchanged throughout gestation (0.18 ± 0.06), becoming significantly higher during the last weeks of gestation compared to the two other groups (p < 0.001). In fetuses with VSDs, the ISI measurement from the aortic isthmus Doppler flow during the second half of gestation is a reliable predictor of a large non-restrictive defect with risk of major postnatal clinical impact.

The Effect of Levosimendan Versus Milrinone on the Occurrence Rate of Acute Kidney Injury Following Congenital Heart Surgery in Infants: A Randomized Clinical Trial.

It has been shown that, in contrast to other inotropic agents, levosimendan improves glomerular filtration rate after adult cardiac surgery. The aim of this study was to investigate the efficacy of levosimendan, compared with milrinone, in preventing acute kidney dysfunction in infants after open-heart surgery with cardiopulmonary bypass. Two-center, double-blinded, prospective, randomized clinical trial. The study was performed in two tertiary pediatric centers, one in Sweden (Gothenburg) and one in Finland (Helsinki). Infants between 1 and 12 months old, diagnosed with Tetralogy of Fallot, complete atrioventricular septal defect or nonrestrictive ventricular septal defect, undergoing total corrective cardiac surgery with cardiopulmonary bypass. Seventy-two infants were randomized to receive a perioperative infusion of levosimendan (0.1 µg/kg/min) or milrinone (0.4 µg/kg/min). The infusion was initiated at the start of cardiopulmonary bypass and continued for 26 hours. The primary outcome variable was the absolute value of serum creatinine data on postoperative day 1. Secondary outcomes included the following: 1) acute kidney injury according to the serum creatinine criteria of the Kidney Diseases: Improving Global Outcomes; 2) acute kidney injury with serum creatinine corrected for fluid balance; 3) plasma neutrophil gelatinase-associated lipocalin; 4) cystatin C; 5) urea; 6) lactate; 7) hemodynamic variables; 8) use of diuretics in the PICU; 9) need of dialysis; 10) length of ventilator therapy; and 11) length of PICU stays. There was no significant difference in postoperative serum creatinine between the treatment groups over time (p = 0.65). The occurrence rate of acute kidney injury within 48 hours was 46.9% in the levosimendan group and 39.5% in the milrinone group (p = 0.70). There were no significant differences in other secondary outcome variables between the groups. Levosimendan compared with milrinone did not reduce the occurrence rate of acute kidney injury in infants after total corrective heart surgery for atrioventricular septal defect, ventricular septal defect, or Tetralogy of Fallot.

Midterm results of "treat and repair" for adults with non-restrictive ventricular septal defect and severe pulmonary hypertension.

A non-restrictive ventricular septal defect (VSD) can cause intracardiac left to right shunt, which leads to increased pulmonary vascular resistance (PVR) and pulmonary hypertension causes bi-directional or even right-left shunt, namely the Eisenmenger's syndrome. For patients with non-restrictive VSD with severe pulmonary hypertension at stage of near or to be Eisenmenger's syndrome, traditional VSD repair carries high mortality and poor prognosis. Recently, targeted drug therapy was used to decrease pulmonary circulation resistance in these patients before they receive defect repair surgery, namely "treat and repair" strategy, however, there is few reports about the midterm result of this strategy in adults with non-restrictive VSD with severe pulmonary hypertension at stage of near or to be Eisenmenger's syndrome. In this study, we used this strategy to treat 41 adult VSD patients who received bosentan as the targeted therapy to decrease their PVR before and after repair surgery. A total of 39 patients were followed up for an average of 37 months. None of the patients died during follow-up. Among them, 36 cases continued targeted drug therapy, whose mean pulmonary artery pressure (mPAP) was significantly reduced, including 31 cases with mPAP <50 mmHg, and the valve of tap hole was closed. Besides, the SpO2 was significantly elevated. These results demonstrated that "treat-and-repair" strategy may be a viable approach for the adults with non-restrictive VSD with severe pulmonary hypertension at stage of near or to be Eisenmenger's syndrome.

Transversus abdominis plane block as sole anaesthetic technique for inguinal hernia repair in two patients having complex medical conditions

Sir, Transversus abdominis plane (TAP) block has been used as a sole anaesthetic technique for the patients with multiple co-morbidities presenting for emergency laparotomy.[1,2] We share our experience of using TAP block for the surgical anaesthesia in two patients scheduled for inguinal hernioplasty who had associated complex medical conditions. First patient was a 52-year-old male, known case of congenitally corrected transposition of great arteries (CCTGA) associated with severe pulmonary stenosis and a pressure gradient of 64 mm Hg, large non-restrictive ventricular septal defect (VSD) and right ventricular (i.e., physiologic left ventricle) dysfunction. He was stable on treatment with tablet torsemide. Second case was a 54-year-old male known case of Takayasu's arteritis (TA) and hypertension on treatment with tablet metoprolol and losartan. The patient had the history of claudication and weakness of left upper limb. The peripheral pulses in left upper limb were feeble and blood pressure (B.P) was lower compared to right upper limb (112/70 mm Hg vs. 172/73 mm Hg). For surgery, ultrasound guided (USG) TAP block was administered in both cases using in-plane posterior approach with 20 ml of 0.5% bupivacaine and 10 ml of 2% lignocaine. For visceral analgesia, the TAP block was supplemented with infusions of dexmedetomidine at the loading dose of 1 μg/kg over 10 min followed by 0.5-0.6 μg/kg/min infusion and fentanyl at 75-100 μg/h, respectively, in the first and the second case. In the first patient, T10 -L1 dermatomes were blocked whereas in second patient L1 was spared; only T10 -T12 blockade was achieved as checked by pin prick. The incision line was infiltrated with 2% lignocaine in the second case before surgery. The anaesthetic monitoring in both cases included continuous electrocardiogram, continuous invasive B.P monitoring, pulse oximetry, and temperature. The intraoperative vital parameters were stable and surgery was completed uneventfully in both cases. CCTGA is an uncommon congenital cardiac anomaly characterized by TGA along with the inversion of both ventricles and thus there is both atrio-ventricular and ventriculo-great vessel discordance. The anaesthetic haemodynamic goals in these cases are to maintain systemic vascular resistance (SVR) to avoid right to left shunt by VSD and to preserve myocardial contractility.[3] TA involves a focal stenotic process in the aorta and proximal segment of its main branches. In our second case, though angiography was not done, based on the history of claudication and findings of feeble pulses and decreased B.P. in left arm compared to right is suggestive of stenotic area to be in the arch of aorta. These patients may not tolerate the sudden fall in SVR as they have compromised regional circulation and hence similar to the first patient our intraoperative haemodynamic goal was to maintain SVR. We decided to use USG TAP block in both cases as being an abdominal field, it holds the promising advantage of maintaining the haemodynamics of patients, which are altered in general or neuraxial anaesthesia and additionally provides an excellent postoperative analgesia for 24-48 h. We chose dexmedetomidine infusion in the first patient as it has the combination of analgesic, sedative, and anxiolytic properties with the preservation of respiratory functions. There are 3 main issues which need consideration while using TAP block as a sole anaesthetic modality for surgery. First, TAP block covers only the somatic component of surgical pain; for visceral pain analgesia has to be supplemented with other analgesic agents hence it cannot be a sole anaesthetic technique. Second, the extent of dermatomal block provided by TAP block is uncertain. Both pinprick using blunt needle and cold disinfectant swabs can be used to assess dermatomal extent of sensory blockade. Lee et al.[3] reported that posterior approach produced a median sensory block of three dermatomal segments, the most cephalad being T10. We found the L1 dermatome to be spared in the second case despite using 30 ml of drug for block. Since the incision for inguinal hernia repair lies over T12 -L1 segments, additional infiltration of local anaesthetics (L.A) was required before surgery. Niraj et al.[4] also observed that posterior approach TAP block usually spares L1 segment. Another disadvantage of TAP block is potential to produce L.A. toxicity since large volumes of L.A. are used. There are case reports[5] of cardiac arrests due to L.A. systemic toxicity following TAP block. Therefore the volume of drug used should be carefully determined and the patient should be observed for at least 45 min after TAP block institution during intraoperative and postoperative period. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

PS-297a Catch-up-growth In Term And Preterm Infants After Surgical Closure Of Ventricular Septal Defect In The First Year Of Life: Only Good News?

Failure to thrive is common in children with non-restrictive ventricular septal defect (VSD). Normalisation of growth has been reported after early surgical correction. However the literature is inconsistent about growth velocity after surgery in term and preterm infants. Objective Establishing the pattern of catch-up growth in infants submitted to VSD surgical repair before 1 year of age, for term and preterm infants. Methods 52 infants (41 term, 11 preterm) were studied. Anthropometric data at birth, at time of surgery and 3, 6, 12 and 24 months after surgery, collected retrospectively, were converted to z-scores. Statistic analyses was performed in SPSS® version 21, α=0,05. Results Mean weight and height z scores at the time of surgery were significantly lower for term infants (-2,24 and -1,42, respectively; p Conclusions Early surgical repair of VSD leads to a significant acceleration of growth, mainly in the first 3–6 months after surgery. An increased weight gain velocity has been associated with higher cardiovascular risk later in life. Knowledge of this specific catch-up growth pattern is important and should influence nutritional goals after surgery.

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.

Midterm results of diagnostic treatment and repair strategy in older patients presenting with nonrestrictive ventricular septal defect and severe pulmonary artery hypertension

Background Congenital heart disease with severe pulmonary arterial hypertension (SPAH), previously thought to have irreversible pulmonary vascular disease (PVD), has been recently successfully corrected using diagnostic treatment and repair strategy by our surgery team. This study aimed to evaluate the midterm results of a selected cohort of older patients with nonrestrictive ventricular septal defect (VSD) and SPAH using diagnostic treatment and repair strategy. Methods The records of 56 patients older than six years with nonrestrictive VSD and SPAH undergoing diagnostic treatment and repair strategy from 2006 to 2012 were retrospectively reviewed. All patients received advanced pulmonary arterial hypertension (PAH) therapy and radical repairs were performed when transcutaneous oxygen saturation (SPO2) increased up to 93%. Results There were no operative deaths. SPO2 and baseline six-minute walk test (SMWT) distance of all selected patients increased significantly and mean pulmonary artery pressure (MPAP) regressed significantly after diagnostic treatment and at late follow-up (P&lt;0.01). The incidence of late postoperative PAH was seen in six (10.7%) patients and by Logistic regression analysis, early postoperative PAH was an independent risk factor related to late postoperative PAH. Conclusions Diagnostic treatment and repair strategy was effective and safe for treatment of nonrestrictive VSD and SPAH and the midterm results were excellent. Diagnostic treatment strategy was effective in assessing the reversibility of SPAH in older patients associated with nonrestrictive VSD and the PVD in these selective patients is generally reversible.

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary arterial hypertension associated with congenital heart disease

Congenital heart disease (CHD) with intracardiac/extracardiac shunts is an important etiology of pulmonary arterial hypertension (PAH). The majority of children with congenital cardiac shunts do not develop advanced pulmonary vasculopathy, as surgical repair of the anomalies is now performed early in life. However, if not repaired early, some defects will inevitably lead to pulmonary vascular disease (truncus arteriosus, transposition of the great arteries associated with a ventricular septal defect (VSD), atrioventricular septal defects remarkably in Down syndrome, large, nonrestrictive VSDs, patent ductus arteriosus and related anomalies). The majority of patients are now assigned to surgery based on noninvasive evaluation only. PAH becomes a concern (requiring advanced diagnostic procedures) in about 2-10% of them. In adults with CHD, the prevalence of advanced pulmonary vasculopathy (Eisenmenger syndrome) is around 4-12%.[1]This article will discuss the diagnostic and management approach for PAH associated with CHD (PAH-CHD).

Myocardial cardiotrophin-1 is Differentially Induced in Congenital Cardiac Defects Depending On Hypoxemia

Cardiotrophin-1 (CT-1) is upregulated by hypoxemia and hemodynamic overload and is characterized by potent hypertrophic and protective properties on cardiac cells. This study aimed to investigate whether CT-1 is differentially induced in the myocardium of infants with congenital cardiac defects depending on hypoxemia. Infants with Tetralogy of Fallot (n = 8) or with large nonrestrictive ventricular septal defect (n = 8) undergoing corrective surgery were investigated. Expression of CT-1 was assessed at mRNA and protein levels in the right atrial and ventricular myocardium. The activation of the STAT-3 and VEGF were measured. Degradation of cardiac troponin-I served as a marker of myocardial damage. CT-1 was detected in all patients with levels negatively correlating to the arterial oxygen saturation. Higher CT-1 expression in Tetralogy of Fallot patients was associated with activation of the JAK/STAT pathway and higher cardiac troponin-I degradation. CT-1 may mediate myocardial hypertrophy and dysfunction in infants with congenital cardiac defects, particularly in those with hypoxemia.

Criss-cross heart: Twisted anatomy by cardiovascular magnetic resonance

h o s c D a v A 27-year-old male with known congenitally corrected transposition of the great arteries was referred for further workup due to increasing arrhythmias. Cardiovascular magnetic resonance (CMR) showed situs solitus and atrioventricular (AV) and ventriculoarterial (VA) discordance, with the aorta lying anteriorly and to the right of the pulmonary trunk (Figure 1). An unusual alignment was found, with the right and left ventricular inflow tracts aligned almost perpendicular to one another, precluding imaging all four chambers in one plane. The systemic right ventricle was relatively hypoplastic and lay superior to the subpulmonary left ventricle. The findings were typical of a previously unnoticed criss-cross atrioventricular arrangement. A large nonrestrictive ventricular septal defect and severe pulmonary stenosis were also present;

Early Repair of Large Infant Ventricular Septal Defect Despite Respiratory Syncytial Virus–Induced Respiratory Failure With Postrepair Chylous Pericardial Effusion Requiring Pleuropericardial Window

The surgical correction of congenital cardiac lesions that are complicated by intercurrent respiratory syncytial virus (RSV) pneumonitis has traditionally been deferred for at least 6 to 8 weeks. The presumption is that using cardiopulmonary bypass will increase the risk of postoperative pulmonary complications. We present an infant who developed acute respiratory failure related to RSV pneumonitis and required urgent mechanical ventilation. Cardiac evaluation revealed a large nonrestrictive ventricular septal defect (VSD), aortic arch hypoplasia, normally functioning bicuspid aortic valve, and hemodynamic instability associated with markedly increased pulmonary blood flow. Separation from mechanical ventilation was unsuccessful preoperatively. He underwent VSD repair with cardiopulmonary bypass less than 4 weeks after initial RSV infection. He was extubated successfully within 72 hours of VSD repair. Approximately 6 weeks postoperatively, he developed a circumferential chylous pericardial effusion of unclear etiology--an exceedingly rare complication of VSD repair in early infancy in a non-Down syndrome patient. The chylous effusion was initially managed unsuccessfully with Portogen/Monogen and a percutaneously placed pericardial drain. Two weeks later, he underwent creation of a pleuropericardial window with successful resolution of the chylous effusion. It is of interest to pediatricians to be able to correctly time the repair of congenital heart disease lesions after RSV infection to minimize post-bypass pulmonary complications and yet avoid morbidity from undue delays in repair. In addition, chylopericardium can occur in infants after VSD repair, and dietary modification and catheter drainage may not be adequate.

Quality of Life after Children Undergo a Radical Arterial Switch Operation at an Older Age

The goal of the study was to evaluate the quality of life of children after the older corrective arterial switch operation (ASO) by means of the Pediatric Quality of Life Inventory (PedsQL), version 4.0. The records of 86 patients who had complete transposition of the great arteries plus a nonrestrictive ventricular septal defect, or a Taussig-Bing anomaly, and severe pulmonary arterial hypertension, and who underwent a corrective ASO at an older age (>6 months) between May 2000 and October 2008 were reviewed retrospectively. Eighty survivors were followed up, and the health-related quality of life of the survivors was evaluated with the PedsQL, version 4.0. There were 6 hospital deaths. The mean (SD) follow-up interval was 3.5 ± 2.3 years, and the mean age at last visit was 7.0 ± 1.2 years. Two late deaths occurred, and 8 patients were lost to follow-up. Patients who underwent a corrective ASO at an older age showed acceptable scores for all scales, and they were all comparable with those of a healthy population. Our data suggest that the quality of life of children who undergo a corrective ASO at an older age (>6 months) is acceptable, compared with that of healthy children in China.

Unoperated Congenitally Corrected Transposition of the Great Arteries, Nonrestrictive Ventricular Septal Defect, and Pulmonary Stenosis in Middle Adulthood

Submitted May 6, 2011; Accepted August 3, 2011. The survival into adulthood of patients with unoperated complex congenital heart disease with anomalies often considered life threatening in infancy and childhood requires a complex interplay of "balanced" defects allowing for cardiovascular physiology compatible with long-term survival. We report on a series of three cases from our advanced imaging database of middle-aged adults presenting with multiple similar defects providing a hemodynamically balanced circulation. The constellation of defects seen in each of these patients included congenitally corrected transposition of the great arteries, a large nonrestrictive ventricular septal defect, valvular pulmonary stenosis, and in two cases anomalous coronary arteries. Cardiovascular computed tomographic angiography (CCTA) and cardiovascular magnetic resonance imaging (CMR) were important to the characterization of the multiple defects and their three-dimensional relationships in these cases. Treatment decisions in patients with this constellation of findings are challenging, given the limited data due to the rarity of survival of patients with these defects into middle adulthood and the paucity of data related to decisions and approaches to medical management, surgical correction, or transplantation.

Midterm Results of Arterial Switch Operation in Older Patients With Severe Pulmonary Hypertension

It is widely accepted that patients older than 6 months with dextrotransposition of the great arteries and nonrestrictive ventricular septal defect or Taussig-Bing anomaly and severe pulmonary hypertension usually are not candidates for an arterial switch operation or even repair. This study aimed to evaluate the midterm results of the arterial switch operation in these selected patients. The records of 86 patients older than 6 months with dextrotransposition of the great arteries and nonrestrictive ventricular septal defect or Taussig-Bing anomaly and severe pulmonary hypertension undergoing the arterial switch operation at our institution from 2000 to 2008 were reviewed retrospectively. Eighty survivors were followed. There were 6 hospital deaths (7.0%, 95% confidence interval, 1.6% to 12.4%). From 2006 to 2008, 46 were operated without deaths occurring. The median duration of follow-up was 42 months. Two late deaths occurred. Latest follow-up data showed that 2.8% of survivors were in New York Heart Association class II and 97.2% in class I. Midterm results of the arterial switch operation for patients older than 6 months with dextrotransposition of the great arteries and nonrestrictive ventricular septal defect or Taussig-Bing anomaly and severe pulmonary hypertension are excellent in the current era.