Mitral and Aortic Atresia Associated with Hypoplastic Right Lung, Crossover Segment of Right Lower Lobe, and Anomalous Scimitar-like Right Pulmonary Venous Connection with Inferior Vena Cava: Clinical, Angiocardiographic, and Autopsy Findings in a Rare Case

A newborn female infant was found to have a unique and previously unreported group of anomalies: (1) mitral and aortic atresia with a highly obstructive atrial septum; (2) hypoplasia of the right lung with a crossover segment involving the right lower lobe; (3) normally connected pulmonary veins, two from the left lung and one from the right; and (4) a large anomalous branch of the right pulmonary vein of scimitar configuration that anastomosed with the normally connected right pulmonary vein and with the inferior vena cava (IVC). The scimitar vein appeared obstructed at its junction with the right pulmonary vein and at its junction with the inferior vena cava within the hepatic parenchyma. To our knowledge, this is the first report of a scimitar-like vein coexisting with mitral and aortic atresia and connecting both with the right pulmonary vein and with the inferior vena cava. The highly obstructed left atrium was partially decompressed by retrograde blood flow via the normally connected right pulmonary vein to the anomalous scimitar venous pathway and thence to the inferior vena cava via a pulmonary-to-IVC collateral vein. Received March 18, 1997; accepted August 6, 1997.     

Treatable cardiomyopathies

Cardiomyopathy is defined as primary myocardial dysfunction which is not due to hypertensive, valvular, congenital, coronary or pulmonary vascular disease. This term usually denotes a dismal prognosis short of cardiac transplantation. However, several organic diseases of the heart can result in right or left ventricular dysfunction resulting in congestive heart failure and prompting the physician to label them as cardiomyopathy; the etiological factor is overlooked as it produces very subtle features. Therefore, before labelling any child as cardiomyopathic, all possible causes of ventricular dysfunction must be excluded by clinical and investigative means. The causes of “treatable cardiomyopathy” include mechanical factors as critical aortic stenosis and pulmonic stenosis, severe coarctation of aorta in an infant and aortaarteritis is an older child. Some of the persistent arrhythmias like atrial tachycardia, fibrillation, paroxysmal junctional re-entrant tachycardia are also known for causing ventricular dysfunction producing tachycardiomyopathy. Treatment of arrhythmia improves the ventricular function. Myocardial ischemia as a result of congenital coronary anomaly (commonest being anomalous origin of left coronary artery from pulmonary artery) can also present with a cardiomyopathy like picture. Early surgical correction is very rewarding. Finally, some of the metabolic conditions like creatinine and thiamine deficiency can also produce ventricular dilatation and dysfunction. In conclusion, the so called cardiomyopathy like picture can be produced because of several reasons and an attempt must be made to identify them.     

复杂性先天性心脏病的介入治疗

目的探讨先天性心脏病(简称先心病)介入治疗在复杂性先心病中的应用.方法应用Rashkind球囊导管对59例复杂性先心病患儿行球囊房隔造口术(BAS);对2例右室发育不良综合征患儿进行静态球囊房隔扩张造口术;对1例在大动脉转位术后出现肺动脉吻合口狭窄的完全性大动脉转位(TGA)的患儿,采用球囊扩张导管进行扩张;对15例法洛四联症(TOF)患儿进行经皮球囊肺动脉瓣成形术(PBPV)、球囊血管成形术;对18例合并主动脉至肺动脉侧支血管的患儿以及5例B-T分流术的患儿在外科根治术前采用弹簧圈进行侧支血管和分流管道的堵塞术;对1例Fontan手术后的先心病患儿采用Amplatzer房间隔缺损(简称房缺)堵塞装置进行窗孔堵塞术.结果46例TGA患儿BAS后动脉血氧饱和度(SaO2)由0.57±0.17上升至0.76±0.13(t=14.58, P<0.01),左右心房压差﹤2 mmHg(1 mmHg=0.133 kPa),术后房缺5～20 mm;10例肺动脉闭锁伴室间隔完整的患儿BAS后SaO2变化不大(t=1.57, P﹥0.05),左右心房压差﹤2 mmHg;TOF患儿经PBPV及球囊血管成形术后SaO2平均上升15%,肺动脉瓣狭窄解除;18例主动脉至肺动脉侧支血管堵塞术,14例效果良好被完全堵塞;5例B-T分流管道堵塞术,全部封堵成功;1例Fontan手术后窗孔堵塞术,获完全封堵.结论复杂性先心病应用介入治疗和外科手术相配合,可获得更好的疗效.     

Cardiac atrial natriuretic peptide in monocrotaline-induced pulmonary hypertensive rats

To determine the time course and potential triggers for synthesis of atrial natriuretic peptide (ANP) in right ventricle during the development of right ventricular hypertrophy (RVH), we measured mean right atrial pressure, right ventricular systolic pressure (RVSP), immunoreactive ANP (iANP) concentrations in plasma, and cardiac iANP concentrations and contents of monocrotaline (MCT)-induced pulmonary hypertensive rats treated with a subcutaneous injection of MCT (40 mg/kg). Following the development of RVH and pulmonary hypertension, iANP concentrations in plasma and iANP concentrations and contents in ventricular tissues increased with time. At the third week after treatment, iANP concentrations in MCT rats increased 6-fold in plasma, 57-fold in the right ventricular free wall, 20-fold in the ventricular septum and 10-fold in the left ventricular free wall compared with those in controls. At the third week, iANP contents of total ventricular tissues in MCT and control rats were 29% and 0.8%, respectively, of the corresponding atrial contents. The iANP concentrations and contents of right ventricular free walls in MCT rats were greater than those of any other parts of ventricular tissues. The iANP concentrations in right ventricular free walls were very closely related to RVSP (r = 0.93, P < 0.001). In MCT rats, iANP concentrations in right atrial tissues decreased with time. This study shows that ventricular ANP contributes to the amount of ANP stored within the heart in MCT rats and that pressure overload may be one of the triggers for ANP synthesis in the right ventricle.     

Double total anomalous pulmonary venous connection

We present a newborn infant with right atrial isomerism, complex congenital heart malformation and anomalous pulmonary venous connection, reliably diagnosed by 2-D Doppler color echocardiography. The infant had no significant obstetric antecedents. The neonatal clinical picture included cyanosis, heart murmur and respiratory distress. The infant was treated with prostaglandin from the age of 24 hours until his death after surgery. The 2-D echo Doppler color flow mapping showed findings that suggested right atrial isomerism, severe left ventricular hypoplasia, pulmonary atresia and ductus arteriosus. The pulmonary veins flowed together to a posterior cardiac chamber from which an emissary vertical venous vessel connected with a left superior aneurysmal sack. Two venous channels emerged from this sack: one connecting to the innominate vein and the other to the atrium. The malformations were confirmed by cardiac catheterization. On the sixth day of life, the patient underwent anastomosis between the posterior venous chamber with the atrium, a modified Blalock-Taussig shunt implant, and ductus closure but died during surgery. The association between complex cardiac anomalies and uncommon obstructive total anomalous pulmonary venous connection in the context of right atrial isomerism is lethal and few neonates survive surgical repair. Two-dimensional echo color flow Doppler is a reliable diagnostic technique and an indispensable guide in angiography.     

Electrophysiologic Studies and Radiofrequency Catheter Ablation of Ectopic Atrial Tachycardia in Children

Ectopic atrial tachycardia (EAT) often resists medical therapy, making radiofrequency catheter ablation (RFCA) the preferred treatment. This study reviewed the records of 35 patients who underwent electrophysiologic studies (EPS) and 39 RFCA procedures for EAT during a 10-year period. Of the 35 patients, 10 (28%) presented with decreased ventricular function and tachycardia-induced cardiomyopathy (TIC). The EAT originated on the right atrial side in 19 patients (54%) and on the left atrial side in the remaining 16 patients (46%). The right atrial sites included the right atrial appendage (RAA) (n = 9, 25%), the tricuspid annulus (n = 7, 20%), and the crista terminalis (n = 3). The left atrial sites included the left atrial appendage (LAA) (n = 6, 17%), the pulmonary veins (n = 5, 14%), the mitral annulus (n = 3), and the posterior wall of the left atrium (n = 2). The mechanism of all EAT probably is automaticity. All EATs could be abolished using RFCA. Follow-up data were available for all patients 2 to 8 years after RFCA. All 35 patients remained recurrence free, and ventricular function improved for all 10 patients with TIC. The origin of EAT in children differed from its origin in adults. The authors conclude that RFCA is a safe and effective treatment option for children with refractory EAT and should be considered early in the course of their illness.     

Hemodynamic effects of sodium nitroprusside in patients with ventricular septal defect

During routine cardiac catheterization of ten patients with ventricular septal defect, the hemodynamic parameters were measured in the resting state and during sodium nitroprusside infusion at an average rate of 8 g/kg/min. Two distinct hemodynamic groups could be identified. Group I characterised by elevated left ventricular filling pressure showed a consistently favourable response to sodium nitroprusside infusion with a decrease in the left ventricular filling pressure, the pulmonary arterial pressures and the left to right shunt. The favourable effect was most pronounced in patients who had pulmonary arterial hypertension in addition to elevated left ventricular filling pressures. Group II patients characterised by normal left ventricular filling pressures showed a variable response to sodium nitroprusside administration. The left ventricular end-diastolic pressure fell or was unaltered. The pulmonary arterial pressures and pulmonary vascular resistance fell in those with pulmonary arterial hypertension. Patients without pulmonary arterial hypertension showed an increase in the magnitude of left to right shunt. On the basis of our data, we feel that vasodilator drugs have a specific role in the management of congestive heart failure secondary to large left to right shunts. The present study strongly indicates the need for further investigations utilizing orally effective vasodilators in patients with congestive heart failure due to left to right shunts.     

A Single-Center Experience with Intracardiac Thrombosis in Children with Dilated Cardiomyopathy

Intracardiac thrombosis in patients with a dilated cardiomyopathy can be life threatening. This study investigated the incidence, risk factors, and outcome of intracardiac thrombosis in children with dilated cardiomyopathy. A retrospective review of the clinical records was performed in 83 children with dilated cardiomyopathy diagnosed from January 1995 to December 2008. Intracardiac thrombi were detected in 5 patients (6.0%). The intracardiac thrombi were found mainly in the left ventricle (n = 3). One patient had a thrombus in the left atrium at the time of diagnosis, and a right ventricular thrombus was found in 1 patient with unrepaired ventricular septal defect complicated by pulmonary hypertension. Intracardiac thrombosis developed during rapid deterioration of ventricular function, and all patients had a poor ejection fraction of the left ventricle. All patients were treated with heparinization, and thrombectomy was performed in 1 patient. Three patients achieved complete resolution of the thrombus without further embolic complications. Careful evaluation and aggressive anticoagulation are necessary for the prevention of intracardiac thrombosis in children with poor ventricular function, especially during rapid deterioration of ventricular function.     

Plasma and right auricle concentrations of atrial natriuretic polypeptide in children with cardiac diseases

To investigate the atrial natriuretic polypeptide (ANP) levels in congenital heart diseases, we measured plasma immunoreactive ANP (ir-ANP) in 29 patients with left-to-righ shunt, and right auricle ir-ANP in 25 patients who underwent operation. The levels of ir-ANP in the pulmonary artery correlated with that in the femoral vein (r=0.95, P<0.001), mean pulmonary arterial pressure (r=0.74, P<0.001) and right atrial pressure (r=0.53, P<0.01). The right auricle ir-ANP level was high in patients with heart failure combined with pressure or volume overload of the right atrium, as observed in tricuspid atresia, complete endocardial cushion defect, and total anomalous pulmonary venous drainage. These results suggest that the production of ANP in the atrium increases as a compensatory or secondary response to persistent release of ANP into the circulation in cardiac diseases.Abbreviations ANP atrial natriuretic polypeptide - ir-ANP immunoreactive ANP - -hANP alpha human ANP - VSD ventricular septal defect - ASD atrial septal defect - PDA patent ductus arteriosus - TOF tetralogy of Fallot - DORV double outlet right ventricle - TAPVD total anomalous pulmonary venous drainage - cECD complete endocardial cushion defect - TA tricuspid atresia - MRAP mean right atrial pressure - MPAP mean pulmonary arterial pressure - Qp/Qs pulmonary-to-systemic blood flow ratio     

Symptomatic atrial septal defect in the neonate and infant.

Atrial septal defect, secundum type, is rarely diagnosed and seldom produces symptoms in infancy. 7 patients, aged between 2 weeks and 8 months, who developed congestive failure, are presented. 6 of them had an atrial LR-shunt documented at cardiac catheterization. 1 diagnosis was made at post mortem in the only infant who died. The clinical signs of an ASD do not vary from those normally found in the older child; the causes for early failure are probably rapidly decreasing pulmonary vascular reistance and disturbances of left ventricular compliance. 6 infants improved with conservative or no treatment, although spontaneous closure or significant diminution of the shunt did not occur. Surgical closure can be deferred to a later optimal age for operation. ASD secundum type should be considered as a cause of heart failure in the neonate and infant.     

Atrial natriuretic peptide concentrations in children with pulmonary hypertension: Correlation with hemodynamic measurements

Summary To define the relation between atrial natriuretic peptide (ANP) and hemodynamic parameters in children with pulmonary artery hypertension, we measured the ANP concentrations of the femoral vein, right atrium, pulmonary artery, left atrium and left ventricle, or femoral artery in 32 patients during right or left heart catheterization. There is a strong correlation among the ANP levels obtained from different locations (p<0.001), and these ANP levels are positively correlated with pulmonary arterial pressures and pulmonary resistance. Patients were divided into two groups according to their pulmonary arterial pressure. The group with pulmonary arterial systolic pressure higher than 31 mmHg had higher ANP levels than the group with pulmonary arterial systolic pressure lower than 31 mmHg. When patients were grouped according to their pulmonary resistance, ANP levels in the group with pulmonary resistance over 2 U/m² were higher than those in the group with pulmonary resistance lower than 2 U/m². In this study ANP levels showed a correlation with the right ventricular systolic pressure. A correlation was not seen between ANP levels and the flow ratios or the presence of shunt. The results of our study suggest that ANP should be considered an important factor in pulmonary hypertension, independent of other factors.     

Stepwise interventional approach in a neonate with pulmonary valve atresia and intact ventricular septum

In neonates with pulmonary atresia and intact ventricular septum the aims of therapy are maintenance of pulmonary blood flow and right ventricular decompression in order to achieve right ventricular support of the pulmonary circulation. Recent developments in interventional heart catheterization with pulmonary radiofrequency-assisted balloon valvuloplasty and ductal stent implantation offer an alternative to the classical surgical approach. We report on a neonate with membranous pulmonary atresia and intact ventricular septum, in whom a large interatrial right-to-left shunt via the foramen ovale persisted after radiofrequency-assisted pulmonary balloon valvuloplasty on the 2nd day of life. The interatrial shunt prevented adequate right ventricular filling and antegrade pulmonary perfusion leading to severe cyanosis (transcutaneous oxygen saturation 40%). In order to increase pulmonary blood flow and raise left atrial pressure, the arterial duct was stented. After ductal stenting, prostaglandin was discontinued and the transcutaneous oxygen saturation remained stable around 89%. At follow up after 7 weeks the foramen ovale had decreased in size and only a small left-to-right shunt was present, documenting the effectiveness of this approach. Conclusion Based on the present case we propose a stepwise interventional approach for the neonate with pulmonary atresia and intact ventricular septum. If cyanosis persists after isolated pulmonary valvuloplasty despite adequate right ventricular function, ductal stent implantation can reduce interatrial shunting and thus improve oxygen saturation. Received: 12 January 1998 / Accepted: 15 February 1998     

Neonatal Pulmonary Hypertension: Pathophysiologic-Based Therapy

Rational management of pulmonary artery hypertension (PAH) in the newborn infant must be based on an understanding of underlying causes. When a specific diagnosis can be made, empirical treatment gives way to specific therapy. For every pathophysiologic abnormality potentially resulting in PAH, clinical entities can be identified and key therapeutic interactions can be considered. 1. Pulmonary Venous Hypertension: PAH is secondary to obstruction of blood flow through the left heart. If based on anatomic abnormality (mitral or left atrial), surgical relief of obstruction is required. If PAH is the result or functional abnormality of the left ventricle (transient left ventricular ischemia), medical treatment of left ventricular failure is most important. 2. Functional Obstruction of Pulmonary Vascular Bed: Hyperviscosity of blood (polycythemia - HCT > 65) results in increased pulmonary vascular resistance. Treatment consists of reduction of red blood cell volume. 3. Pulmonary Vascular Constriction: Hypoxemia with or without pulmonary parenchymal disease (primary or secondary persistent fetal circulation [PFC] syndrome). Therapy is directed at improvement of oxygenation and dilatation of pulmonary arteriolar bed (e.g. O₂ hyperventilation and/or pulmonary vasodilator therapy). 4. Decreased Pulmonary Vascular Bed: If primary pulmonary hypoplasia is present, no treatment is available. If secondary form (diaphragmatic hernia), surgical repair and pulmonary vasodilatation of contralateral lung is indicated. Extracorporeal membrane oxygenation (ECMO) can be used in severe cases. 5. Increased pulmonary blood flow: Causes in the neonate include complex heart disease without pulmonary stenosis; and peripheral A—V fistula. Treatment: Surgical limitation of pulmonary blood flow may be required. Even when PAH is the result of combined etiologies, a prompt well-organized clinical management plan can follow from understanding of pathologic principles.     

Transcatheter Ventricular Septal Defect (VSD) Creation for Restrictive VSD in Double-Outlet Right Ventricle

Background

Double-outlet right ventricle (DORV) with a restrictive ventricular septum is a rare but highly morbid phenomenon that can be complicated by progressive left ventricular hypertrophy, arrhythmias, aneurysm formation, severe pulmonary hypertension, and death in the newborn. Surgical creation or enlargement of a ventricular septal defect (VSD) is palliative but may damage the conduction system or the atrioventricular valves in the newborn. This report presents a transcatheter approach to palliation for a newborn that had DORV with a restrictive ventricular septum.

Methods/Results

A full-term infant girl (2.9 kg) referred for hypoxia (80 % with room air) and murmur was found to have DORV, interrupted inferior vena cava, and restrictive VSD (95-mmHg gradient). Transhepatic access was performed, and an internal mammary (IM) catheter was advanced through the atrial septal defect and into the left ventricle. By transesophageal echocardiographic guidance, a Baylis radiofrequency perforation wire was used to cross the ventricular septum, and the defect was enlarged using a 4-mm cutting balloon. A bare metal stent then was deployed to maintain the newly created VSD. The patient did well after the procedure but required pulmonary artery banding 4 days later. She returned 5 months later with cyanosis and the development of obstructing right ventricle muscle bundles, requiring further surgical palliation.

Conclusions

This report describes the first transcatheter creation of VSD in DORV with a restrictive ventricular septum in a newborn infant. Use of the radiofrequency catheter in combination with cutting balloon dilation and stent implantation is an efficient method for creating a VSD in such a patient.     

Serial Plasma BNP Levels in Assessing Inadequate Left Ventricular Decompression on ECMO

We describe a female infant with complex single ventricle physiology who had undergone median sternotomy for placement of a right-sided systemic-to-pulmonary artery shunt, division of a patent ductus arteriosus, and left pulmonary artery augmentation. Her early postoperative course was complicated by cardiac arrest requiring institution of extracorporeal membrane oxygenation (ECMO) support. The brain natriuretic peptide (BNP) levels acutely improved after left ventricular decompression by insertion of a left atrial cannula. In this setting BNP levels may be an indicator of left ventricular stretch and are potentially a useful index to monitor left ventricular distension.     

Biventricular Long Axis Function After Repair of Tetralogy of Fallot

Right ventricular restrictive physiology is common after repair of tetralogy of Fallot and relates to exercise performance and symptomatic arrhythmias. In this study, we examined biventricular long axis function in an attempt to clarify further the mechanical substrate of this phenomenon. We studied prospectively 95 patients with tetralogy of Fallot (age range 1–44.3 years) at a median of 4.3 years after repair with Doppler and M-mode echocardiography. Pulmonary arterial, tricuspid, and mitral Doppler spectrals and 2-D guided M-mode recordings of ventricular minor and long axes were obtained with simultaneous phonocardiogram and respirometer recordings. Right ventricular restriction was defined by the presence of antegrade pulmonary arterial flow during atrial systole throughout the respiratory cycle. Restrictive right ventricular physiology was demonstrated in 36 (39%) [group 1] of the 92 patients in whom the data were analyzed. Left ventricular function (FS, isovolumic relaxation time and transmitral E wave deceleration time) was not different in the two groups (p < 0.1, p < 0.6, and p < 0.8, respectively). The presence of antegrade diastolic flow shortened the pulmonary regurgitation in the restrictive group (PR duration/√RR 10.7 ± 2.1 vs 12.1 ± 2.1, p < 0.01). There was delayed onset of shortening (97.4 ± 24 vs 88.8 ± 24 ms, p= 0.01), and the amplitude of right atrioventricular ring excursion, corrected for body surface area, was significantly lower during atrial systole in the restrictive group (0.43 ± 0.15 vs 0.54 ± 0.2 cm/m², p < 0.01). There was also a tendency toward a smaller ratio of right to left total atrioventricular ring excursion in the same group (1.14 ± 0.19 vs 1.22 ± 0.23, p= 0.1). Impaired long axis function in patients with restrictive right ventricular physiology following repair of tetralogy of Fallot is associated with abnormal diastolic filling and may contribute to the long-term cardioprotective effect of restrictive physiology by limiting the degree of right ventricular dilatation.     

Left atrium thrombus in an extremely low‐birthweight infant with late‐onset circulatory dysfunction

A left atrium thrombus, potentially a life‐threatening complication, is an extremely rare in early infancy. Most cases are caused by mal‐placement of central venous catheters or related to congenital heart diseases with left atrial blood congestion. Here we present an extremely low birth weight infant who developed a left atrial thrombus during the course of late onset circulatory dysfunction. The thrombus was successfully treated by recombinant tissue plasminogen activator. A hemodynamically unstable condition like late onset circulatory dysfunction should be taken into consideration as a potential risk condition of this rare disease.     

Severe Left Ventricular Hypoplasia in Patients with Unbalanced Incomplete Atrioventricular Septal Defect and Pulmonary Hypertension: Feasibility of Biventricular Repair

We report three patients with partial atrioventricular septal defect who presented in the neonatal period with excessive left to right shunting and progressive pulmonary hypertension. Successful biventricular repair was accomplished despite the fact that left ventricular area and left sided structures did not meet the criteria established previously for biventricular management in children with critical aortic stenosis or complete atrioventricular septal defect. Indication for biventricular management was based on the fact that none of our patients had morphological mitral or aortic stenosis and that the hypoplastic left ventricle proved capable to maintain the systemic circulation following closure of the arterial duct. Fenestrated closure of the atrial septum and an individualized approach regarding the closure of the cleft in the left sided atrioventricular valve appear to be important surgical options to reduce postoperative left atrial pressure and to allow the ventricles to adapt to the new loading conditions.     

Plasma Concentrations of Atrial and Brain Natriuretic Peptides and Cyclic Guanosine Monophosphate in Response to Dobutamine Infusion in Patients with Surgically Repaired Tetralogy of Fallot

We examined the plasma concentrations of atrial and brain natriuretic peptides (ANP and BNP) and cyclic guanosine monophosphate (cGMP) during dobutamine infusion and their relationship with hemodynamic parameters in 14 patients with surgically repaired tetralogy of Fallot (TOF). Dobutamine was infused at an initial dose of 5 μg/kg/min and increased by 5 μg/kg/min up to 20 μg/kg/min. The plasma ANP, BNP, and cGMP concentrations were determined before infusion, at the end of each stage, and 15 minutes after discontinuing dobutamine infusion. The plasma concentrations of ANP, BNP, and cGMP were elevated in all patients before dobutamine infusion. The ANP, BNP, and cGMP concentrations decreased in 11 of the 14 patients during dobutamine infusion. In contrast, the plasma ANP and BNP concentrations increased in the remaining 3 patients without a change in the cGMP concentration. The right ventricular pressure and volume were significantly elevated in these patients. The plasma cGMP concentration correlated with the ANP concentration (r= 0.62, p < 0.01) but not the BNP concentration. The plasma ANP concentration during dobutamine infusion correlated with right ventricular systolic pressure (r= 0.71, p < 0.05), mean right atrial pressure (r= 0.29, p < 0.05), and mean pulmonary capillary wedge pressure (r= 0.32, p < 0.05). The BNP concentration correlated with right ventricular volume (r= 0.61, p < 0.05) and systolic pressure (r= 0.46, p < 0.05). In conclusion, rapid changes in ANP, BNP, and cGMP concentrations during dobutamine infusion reflect the changes in atrial and ventricle pressure and volume overload. In surgically repaired TOF, the ANP concentration is affected by right ventricular systolic pressure, right atrial pressure, and pulmonary capillary pressure. Furthermore, the BNP concentration reflects right ventricular pressure and volume overload.     

An Infant with Subvalvar and Valvar Aortic Stenosis,Subvalvar and Valvar Pulmonary Stenosis,Severe Biventricular Hypertrophy and Pulmonary Hemorrhage: A Case Report

An infant with subvalvar and valvar pulmonary stenosis, subvalvar, and valvar aortic stenosis and hypertrophic cardiomyopathy, who presented with pulmonary hemorrhage, is reported. He had right ventricular hypertrophy, thickened pulmonary valve leaflets, severe asymmetric left ventricular hypertrophy with outflow tract obstruction, and a thickened and dysplastic aortic valve.