Normal Pulmonary Valve Research Articles

New insights into the hemodynamics of pulmonary homograft patients under stress echocardiography: The contribution of pressure recovery.

The importance of pulmonary artery pressure recovery (PR) in patients with Ross procedures in whom a homograft substitutes the resected pulmonary valve, is unknown. The aim of the study was to evaluate the occurrence and extent of PR in the pulmonary artery in 65 asymptomatic patients with pulmonary homograft after Ross surgery during rest and exercise. Stress echocardiography was performed in 65 pulmonary homograft patients and 31 controls with native pulmonary valves up to 75W. Right ventricular systolic pressure (RVSP), transvalvular flow, mean pressure gradient (Pmean ), valve resistance, and RV stroke work were determined in the exercise (max. 75W) and recovery phases in increments of 25W each. Pulmonary homografts demonstrated significantly elevated Pmean compared to controls at all stages. When considering pressure recovery (absolute and relative PR at rest 3.8±1.8mm Hg, 42.6±7.2%, respectively) and transvalvular energy loss (EL; at rest 4.5±4.3mm Hg) the homograft hemodynamics reached the level of controls. In a subgroup of patients with tricuspid regurgitation, resting RVSP was the same in homograft patients and controls (21.3±6.1 vs. 20.4±6.3, p=.62), despite significant different Pmax values. Ross patients with pulmonary homograft showed systematically increased hemodynamic parameters compared to normal pulmonary valves. These differences were abolished when PR was considered for homograft patients. The equality of RVSP values at rest in both groups shows non-invasive evidence for PR in the pulmonary system after homograft implantation. Therefore, PR appears to be an important measure in calculating the actual hemodynamics in pulmonary homografts.

Abstract 13878: A Case of Severe Tricuspid Regurgitation, Recurrent Ascites, Worsening Renal Function Leading to Hemodialysis Secondary to RV Lead Impingement, Resolved Completely With Lead Extraction

Introduction: 1. In contemporary practice, implantation of Cardiovascular Implantable Electronic Devices (CIED) is associated with low complication rate peri-procedurally. 2. However, in some patients these complications can have high morbidity and mortality; namely pneumothorax, pericardial tamponade, pacemaker syndrome, and rarely tricuspid regurgitation (TR) secondary to lead impingement. 3. TR secondary to RV lead impingement is not uncommon but infrequently lead to severe TR and worsening heart failure symptoms. 4. We present a case of severe TR secondary to RV lead impingement leading to splanchnic congestion and recurrent ascites, worsening renal failure leading to hemodialysis (HD), all of which resolved with lead extraction. Case: A 77-year-old woman with known CAD with prior PTCA, paroxysmal AF, and prior watchman implantation, evaluated in our clinic for severe TR. She had recurrent admissions for ascites needing paracentesis. Her liver function and ultrasound was normal. Her renal function worsened and HD was initiated. ECHO showed normal LVEF, severe TR with RVSP of 74mmHg, and mild RV dysfunction. Pericardial physiology was normal. The lead was noted to be impinging on the septal leaflet, and adherent. Normal pulmonary valve function. Follow up: We proceeded to perform RV lead extraction and implanted a new leadless pacemaker [MICRA-AV, Medtronic Inc]. Her follow up ECHO in 2 weeks showed mild TR and normal RV function. RVSP had dropped significantly to 34mmHg. Surprisingly, her needs for paracentesis came down and by 3 months follow up she did not need paracentesis anymore. Her renal function improved, and her HD needs stopped. Conclusions: 1. CIED implantations are associated with low complication rate (less than 5% in total) in most patient cohorts but in some patients it can be associated with significant complications increasing their morbidity and sometimes mortality. 2. Severe TR is a well-recognized complication of pacemaker implantation. It led to recurrent ascites needing paracentesis, ESRD requiring HD and multiple hospital admissions and thus increasing the morbidity. 3. It is important to recognize the uncommon complications of such commonly used intracardiac devices for electrophysiologists, cardiologists and PCPs.

A Case of Isolated Pulmonary Valve Endocarditis: The Importance of Repeat Echography and Microbiology in Diagnosis

A 64-year-old man presented to our centre with a one month history of fatigue, exertional dyspnoea, and worsening lower limb oedema on a background of known HFrEF. Following successful diuresis persistently raised inflammatory markers and intermittent fevers prompted investigation for infective endocarditis (IE). Serial blood cultures grew staphylococcus epidermis which were all, unusually, CO2 dependant and likely from a common source. An initial transthoracic echocardiogram (TTE) showed no evidence of endocarditis and normal Tricuspid (TV) and Pulmonary valves (PV). A subsequent transoesophageal echocardiogram (TOE) showed no significant new valvular abnormality.

Association of Pulmonary Valve Morphology Differences With Outcomes in Tetralogy of Fallot Repair With Right Ventricular Outflow Tract Incision.

Background: Current observational studies may not have large samples to investigate the relationship between pulmonary valve (PV) morphology differences and outcomes after complete repair for tetralogy of Fallot (TOF) by right ventricular outflow tract (RVOT) incision. This study aimed to assess the impact of PV morphology differences on outcomes after complete repair for TOF.Methods: This is a retrospective cohort study. Consecutive patients who underwent TOF repair with RVOT incision at Fuwai Hospital from January 2012 to December 2017 were included and compared according to PV morphology differences (unicuspid or bicuspid was abnormal morphology, while the tricuspid valve was normal morphology). The primary outcome was defined as a composite of death, or reintervention, or significant annular peak gradient (APG), or significant pulmonary regurgitation (PR), whichever occurred first. Multivariable Cox model analysis was used to assess the relationships between PV morphology differences and outcomes. Subgroup analysis and Propensity-score analysis were performed as sensitivity analyses to assess the robustness of our results.Results: The cohort included a total of 1,861 patients with primary diagnosis of TOF, with 1,688 undergoing CR-TOF with RVOT incision. The median age was 318 days [interquartile range (IQR): 223–534 days], a median weight of 8.9 kg (IQR: 7.6–10.5 kg) and 60.0% (1,011) were male. Complete follow-up data were available for 1,673 CR-TOF patients with a median follow-up duration of 49 months. Adjusted risks for the primary outcome and significant APG were lower for patients with normal PV morphology at follow up [adjusted hazard ratio (HR): 0.68; 95% CI: 0.46–0.98; adjusted HR: 0.22; 95% CI: 0.07–0.71, respectively]. The trend for the primary outcome during follow-up remained unchanged, even in subgroups and propensity score matching analyses.Conclusions: In this analysis of data from a large TOF cohort, patients with normal tricuspid PVs were associated with a decreased risk of the primary outcome and a lower risk of significant APG, as compared with patients with abnormal unicuspid or bicuspid PVs.

Double-chambered right ventricle masquerading as bipartite right ventricle.

A 10-day-old neonate with pulmonary consolidation was referred for echocardiography to rule out CHD. At first glance, the morphology appeared to be a bipartite right ventricle with normal tricuspid and pulmonary valves. In-depth analysis, however, of the images showed a double-chambered right ventricle, in which the inlet and outlet portions of the right ventricle were isolated from the apical component of the right ventricle, which itself communicated with the left ventricle through a ventricular septal defect. There was a normal pulmonary valve and tricuspid annulus.

Prenatal diagnosis and postnatal course in four fetuses with very rare pulmonary artery anomalies.

Pulmonary artery (PA) anomalies are very rare congenital cardiac malformations, a significant number of which remain unrecognized or misdiagnosed during the prenatal period. We report the prenatal diagnosis and outcome of pregnancy with fetal PA anomalies and discuss the related management issues. We identified four cases of prenatally diagnosed rare PA anomalies that were seen and confirmed in the newborn period by echocardiography and computed tomographic angiography at our center from 2018 to 2020. The course of the pregnancy, perinatal outcome, and the postnatal course in each case were analyzed. Three fetuses were born by repeat cesarean section approximately at 39 weeks of gestation and the other woman delivered vaginally. Of the abnormal origin of the left PA (LPA) in two patients, the first had right PA abnormalities derivating from the ascending aorta, and in the second, the LPA originated from the right PA. Two patients had agenesis of ductus arteriosus (DA), the first was accompanied with tetralogy of Fallot (TOF) and right aortic arch with a normal pulmonary valve, the second patient presented with an Absent Pulmonary Valve syndrome with TOF. Prenatal ultrasonography can be used to correctly diagnose the abnormal origin of the PA branches. Branching of the PA, presence of DA, location of the aortic, and ductal arch by the trachea should be routinely screened in the prenatal anatomic examination and the three-vessel and trachea view can determine the primary clues of PA malformations.

Computational Modeling of Human Bicuspid Pulmonary Valve Dynamic Deformation in Patients with Tetralogy of Fallot

Pulmonary valve stenosis (PVS) is one common right ventricular outflow tract obstruction problem in patients with tetralogy of Fallot (TOF). Congenital bicuspid pulmonary valve (BPV) is a condition of valvular stenosis, and the occurrence of congenital BPV is often associated with TOF. Dynamic computational models of normal pulmonary root (PR) with tri-leaflet and PR with BPV in patients with TOF were developed to investigate the effect of geometric structure of BPV on valve stress and strain distributions. The pulmonary root geometry included valvular leaflets, sinuses, interleaflet triangles and annulus. Mechanical properties of pulmonary valve leaflet were obtained from biaxial testing of human PV leaflet, and characterized by an anisotropic Mooney-Rivlin model. The complete cardiac cycle was simulated to observe valve leaflet dynamic stress/strain behaviors. Our results indicated that stress/strain distribution patterns of normal tri-leaflet pulmonary valve (TPV) and the BPV were different on valve leaflets when the valve was fully open, but they were similar when valves were completely closed. When the valve was fully open, the BPV maximum stress value on the leaflets was 197.2 kPa, which was 94.3% higher than of the normal TPV value (101.5 kPa). During the valve was fully open, the stress distribution in the interleaflet triangles region of the PR was asymmetric in the BPV model compared with that in the TPV model. The geometric orifice area value in the completely opened position of BPV model was reduced 55.6 % from that of the normal PV. Our initial results demonstrated that valve geometrical variations with BPV may be a potential risk factor linked to occurrence of PVS in patients with TOF. Computational models could be a useful tool in identifying possible linkage between valve disease development and biomechanical factors. Large-scale clinical studies are needed to validate these preliminary findings.

Computational Modeling of Human Bicuspid Pulmonary Valve Dynamic Deformation in Patients with Tetralogy of Fallot

Pulmonary valve stenosis (PVS) is one common right ventricular outflow tract obstruction problem in patients with tetralogy of Fallot (TOF). Congenital bicuspid pulmonary valve (BPV) is a condition of valvular stenosis, and the occurrence of congenital BPV is often associated with TOF. Dynamic computational models of normal pulmonary root (PR) with tri-leaflet and PR with BPV in patients with TOF were developed to investigate the effect of geometric structure of BPV on valve stress and strain distributions. The pulmonary root geometry included valvular leaflets, sinuses, interleaflet triangles and annulus. Mechanical properties of pulmonary valve leaflet were obtained from biaxial testing of human PV leaflet, and characterized by an anisotropic Mooney-Rivlin model. The complete cardiac cycle was simulated to observe valve leaflet dynamic stress/strain behaviors. Our results indicated that stress/strain distribution patterns of normal tri-leaflet pulmonary valve (TPV) and the BPV were different on valve leaflets when the valve was fully open, but they were similar when valves were completely closed. When the valve was fully open, the BPV maximum stress value on the leaflets was 197.2 kPa, which was 94.3% higher than of the normal TPV value (101.5 kPa). During the valve was fully open, the stress distribution in the interleaflet triangles region of the PR was asymmetric in the BPV model compared with that in the TPV model. The geometric orifice area value in the completely opened position of BPV model was reduced 55.6 % from that of the normal PV. Our initial results demonstrated that valve geometrical variations with BPV may be a potential risk factor linked to occurrence of PVS in patients with TOF. Computational models could be a useful tool in identifying possible linkage between valve disease development and biomechanical factors. Large-scale clinical studies are needed to validate these preliminary findings.

A geometric model for the human pulmonary valve in its fully open case.

The human pulmonary valve, one of the key cardiac structures, plays an important role in circulatory system. However, there are few mathematical models to accurately simulate it. In this paper, we establish a geometric model of the normal human pulmonary valve from a mathematical perspective in the fully opening case. Based on the statistical data of the human pulmonary valves, we assume that the motions of the three cusps are symmetrical in the cardiac cycle. Thus, we first propose that each cusp is a part of the cylindrical shell according to its structure and physiological feature. The parameters for the pulmonary valve cusps in three-dimensional space are obtained by the fitting functions. We verify the accuracy of our results by comparing the areas of the pulmonary valve and pulmonary valve flap.

Isolated infundibular pulmonary stenosis

A case of isolated infundibular pulmonary stenosis is reported and literature is reviewed. Infundibular pulmonary stenosis is usually a part of tetralogy of Fallot or is associated with ventricular septal defect. Isolated infundibular pulmonary stenosis is rare. Patients may be asymptomatic in spite of significant stenosis. Dyspnea, chest pain, and presyncope or syncope on effort are common symptoms in significant obstruction. Ejection systolic murmur is best audible in the left 3rd intercostal space and increases on inspiration. Additional murmur of tricuspid regurgitation may appear if there is right ventricular failure. Absence of pulmonary valvular click and normal intensity and splitting of the second sound differentiate it from pulmonary valve stenosis. Electrocardiogram shows right ventricular overload. Two-dimensional (2D) echocardiography shows right ventricular hypertrophy with narrowing of infundibulum, normal pulmonary valve, main pulmonary artery, and branch pulmonary arteries. Right ventricle and right atrium may dilate if there is right-sided failure with significant tricuspid regurgitation. Doppler evaluation reveals systolic gradient in the region of infundibulum with no additional gradient at the level of pulmonary valve, main pulmonary artery, or branch pulmonary arteries. Tricuspid regurgitation may be present. 2D echocardiography with Doppler evaluation also excludes other lesions. Angiography is usually not required. Surgery is safe and effective in relieving obstruction. Stent implantation is safe and effective in highly selected patients with discretely narrow circumferential stenotic segment and near-normal-sized peristenotic segment between the pulmonary and tricuspid valves.

Surgical reconstruction of semilunar valves in the growing child: Should we mimic the venous valve? A simulation study

ObjectivesNeither heart valve repair methods nor current prostheses can accommodate patient growth. Normal aortic and pulmonary valves have 3 leaflets, and the goal of valve repair and replacement is typically to restore normal 3-leaflet morphology. However, mammalian venous valves have bileaflet morphology and open and close effectively over a wide range of vessel sizes. We propose that they might serve as a model for pediatric heart valve reconstruction and replacement valve design. We explore this concept using computer simulation. MethodsWe use a finite element method to simulate the ability of a reconstructed cardiac semilunar valve to close competently in a growing vessel, comparing a 3-leaflet design with a 2-leaflet design that mimics a venous valve. Three venous valve designs were simulated to begin to explore the parameter space. ResultsSimulations show that for an initial vessel diameter of 12 mm, the venous valve design remains competent as the vessel grows to 20 mm (67%), whereas the normal semilunar design remains competent only to 13 mm (8%). Simulations also suggested that systolic function, estimated as effective orifice area, was not detrimentally affected by the venous valve design, with all 3 venous valve designs exhibiting greater effective orifice area than the semilunar valve design at a given level of vessel growth. ConclusionsMorphologic features of the venous valve design make it well suited for competent closure over a wide range of vessel sizes, suggesting its use as a model for semilunar valve reconstruction in the growing child.

Outcomes of neonatal Ebstein's anomaly without right ventricular forward flow

ObjectiveIn neonates with Ebstein's anomaly and absent right ventricular forward flow, pulmonary valve morphology is normal or abnormal. Although initial postnatal presentations of these 2 conditions are similar, clinical courses and therapeutic strategies for each category differ greatly. MethodsAmong 29 neonates with Ebstein's anomaly without right ventricular forward flow on initial postnatal echocardiography, 16 had a normal pulmonary valve and 13 had an abnormal pulmonary valve. ResultsDuring the postnatal follow-up of the normal pulmonary valve group, right ventricular forward flow commenced approximately 10 days after birth (1-15 days). The ductus arteriosus was surgically ligated in 3 neonates to facilitate right ventricular forward flow. Biventricular or 1 1/2 ventricular physiology was eventually achieved in 14 patients in the normal pulmonary valve group (14/16, 88%) and 2 patients in the abnormal pulmonary valve group (2/13, 15.3%). With respect to the preoperative echocardiographic findings, the normal pulmonary valve group had a significantly larger pulmonary valve annulus (8.2 ± 1.4 mm in the normal pulmonary valve group and 6.4 ± 1.8 mm in the abnormal pulmonary valve group, P = .002) and smaller cardiothoracic ratio (0.79 ± 0.05 in the normal pulmonary valve group and 0.85 ± 0.07 in the abnormal pulmonary valve group, P = .03). Mild to moderate pulmonary regurgitation was present in all patients (16/16, 100%) in the normal pulmonary valve group, but 3 patients (3/13, 23%) in the abnormal pulmonary valve group also had pulmonary regurgitation. On logistic regression analysis, only pulmonary valve annulus size remained as an indicator of a normal pulmonary valve (P = .03). ConclusionsIn patients with Ebstein's anomaly and absent right ventricular forward flow, large pulmonary valve annulus size indicated a normal pulmonary valve. Patients with a normal pulmonary valve showed better survival and had a higher probability of achieving biventricular hemodynamics.

Tricuspid Atresia 18 Years Post Glenn: Is Fontan Necessary in All Cases?

Tricuspid atresia is a rare cyanotic congenital heart disease, first described by Kreysig (1817), has an incidence of 1% to 5%. It is incompatible with longevity without surgical intervention. Glenn (1958) was successful in using a superior vena cava to right pulmonary artery anastomosis. Fontan (1958) was successful in separating the right and left cardiac circulation; this is the surgical method of choice, with death of 17% by 20 years of age. The Fontan circulation has burdens of multisystem abnormalities, including ventricular dysfunction (72%), dysrhythmias (40%) and renal, hepatic, gastrointestinal and pulmonary complications. The index case is the oldest documented report of tricuspid atresia with rudimentary right ventricle and concordant ventriculo-arterial connections, 18 years post Glen only, from two years of age. She is asymptomatic, of high intellect, never used medications and is now functioning at New York Heart Association (NYHA) I-II, at 20 years of age. Her survival and function was secondary to an innate large atrial and ventricular communication with normal left ventricle, mitral, aortic and pulmonary valves, indicating Fontan may not be needed in all structural anatomic cases of tricuspid atresia.

Biventricular repair in heterotaxy patients.

Heterotaxy patients' hearts may or may not be suitable for biventricular repair depending on anatomy. Even in the subset that are amenable to surgical septation, cardiac anatomy may present multiple difficulties in achieving a satisfactory repair. However, it is also well known that heterotaxy patients are not ideal candidates for univentricular repair. From 2007 until 2012, a total of 20 patients (11 male) with heterotaxy syndrome underwent biventricular repair (left atrial isomerism: 10 and right atrial isomerism: 10) in our center. Their median age at surgery was 40 (range: 3-108) months. Ten patients had dextrocardia. Eleven patients presented with bilateral superior vena cava, three with inferior vena cava (IVC) draining into left atrium, and six with IVC interruption with azygos or hemiazygos continuation. Anomalous pulmonary venous drainage was present in eight patients. One had a common atrium. Atrioventricular septal defect (AVSD) occurred in nine (complete AVSD in seven) patients. Eight patients had double outlet right ventricle (DORV), one had d-transposition of great arteries (d-TGA), and two had congenitally corrected transposition of the great arteries (CC-TGA). Prior palliative procedures included pulmonary artery banding in three patients and left modified Blalock-Taussig shunt in one patient. Complex intra-atrial baffle constructions were required in all patients to direct pulmonary and systemic venous inflow to the appropriate ventricle. Complete AVSDs were corrected using two-patch technique. Intraventricular tunnel repair was done for DORV. Combined atrial and arterial switch was required to rectify abnormal connections in a child with congenitally corrected transposition with normal pulmonary valve, while a Rastelli + Senning was needed in two children with CC-TGA with pulmonary atresia (n = 1) and double outlet of the right ventricle (n = 1). Major early postoperative complications included intestinal gangrene in four patients for which they underwent bowel resection. Two of these patients could not be salvaged. One patient required coiling of aortopulmonary collateral for early postoperative pulmonary hemorrhage. Two patients needed a tracheostomy for prolonged mechanical ventilatory support. Five patients had a pacemaker implanted for complete heart block. There were no instances of atrial baffle stenosis. Median follow-up was 27 (range: 2-46) months. There was one late death secondary to pneumonia. Satisfactory survival outcomes can be achieved in heterotaxy patients who undergo hemodynamically acceptable biventricular repair. Borderline ventricular hypoplasia and trivial atrioventricular valve regurgitation should not be considered as discouraging factors in anatomically suitable heterotaxy patients as it is possible to adopt a two-stage repair in such patients to achieve biventricular repair at a later stage. Anticipating a higher incidence of conduction problems and gut malrotation preemptively can help reduce the morbidity.

Nitric oxide-sensitive pulmonary hypertension in congenital rubella syndrome.

Persistent pulmonary hypertension is a very rare presentation of congenital virus infection. We discuss the case of complete congenital rubella syndrome presenting at echocardiography with pulmonary hypertension that worsened after ductus ligation. Cardiac catheterization showed a normal pulmonary valve and vascular tree but a PAP = 40 mmHg. The infant promptly responded to inhaled nitric oxide while on mechanical ventilation and was later shifted to oral sildenafil. It is not clear whether our observation may be due to direct viral damage to the endothelium or to the rubella virus increasing the vascular tone via a metabolic derangement.

Aortic valve calcification is mediated by a differential response of aortic valve interstitial cells to inflammation

BackgroundAlthough calcific aortic stenosis is common, calcification of the other three heart valves is not. The aortic valve interstitial cell (VIC) has been implicated in the pathogenesis of aortic stenosis. Proinflammatory stimulation of aortic VICs induces an osteogenic and inflammatory phenotypic change. We hypothesized that the VICs of the other heart valves do not undergo these changes. Using isolated human VICs from normal aortic, mitral, pulmonary, and tricuspid valves, our purpose was to compare the osteogenic response to proinflammatory stimulation via toll-like receptor 4 (TLR-4). Materials and methodsAortic, pulmonic, mitral, and tricuspid (n = 4 for each valve type) VICs were isolated from hearts valves explanted from patients undergoing cardiac transplantation. Cells were cultured and grown to confluence in passage 2–6 before treatment with Lipopolysaccharide (LPS) (100–200 ng/mL) for 24 or 48 h. Cells were characterized by immunofluorescent staining. TLR-4 expression was analyzed (immunoblotting, flow cytometry). Bone morphogenetic protein 2 and intercellular adhesion molecule 1 production were determined (immunoblotting). Monocyte chemoattractant protein 1 levels were determined by enzyme-linked immunosorbent assay. Statistics were by Mann–Whitney U test. ResultsTLR-4 stimulation induced bone morphogenetic protein 2 production only in aortic VICs (P < 0.05). Intra-cellular adhesion molecule 1 production and monocyte chemoattractant protein 1 secretion increased in a similar fashion among TLR-4-stimulated VICs from all four valves. ConclusionsProinflammatory stimulation induces an osteogenic phenotype in aortic VICs but not mitral, pulmonic, or tricuspid VICs. We conclude that this differential osteogenic response of aortic VICs contributes to the pathogenesis of calcific aortic stenosis.

Aortic Valve Calcification is Mediated by a Differential Response of Aortic Valve Interstitial Cells to Inflammation

Mechanisms of inflammation have been implicated in the pathogenesis of calcific aortic stenosis. In response to pro-inflammatory stimulation via toll-like receptor 4 (TLR-4), the aortic valve interstitial cell (VIC) undergoes a phenotypic change from that of a myofibroblast to that of an osteoblast-like cell. This osteogenic phenotype is characterized by the production of bone-forming proteins like bone morphogenetic protein 2 (BMP-2) and alkaline phosphatase (ALP). The aortic valve commonly calcifies, while the other three heart valves do so rarely. We therefore hypothesized that mechanisms of inflammation induce an osteogenic phenotype in aortic VICs but not the VICs from the mitral or right-sided heart valves. Using isolated human VICs from normal aortic, mitral, pulmonary and tricuspid valves, our purpose was to examine and compare the osteogenic response to pro-inflammatory stimulation via TLR-4.

The Ross Operation in Adolescents

The pulmonary autograft aortic valve replacement (Ross AVR) is the AVR of choice for children below the teenage years. Recent literature has questioned the durability of the Ross AVR in older children and young adults that present predominantly with aortic regurgitation and a dilated aortic root. At our center, the Ross AVR has been an excellent choice for most children and young and middle-aged adults. The Ross AVR is an especially good choice in young females who plan on becoming pregnant. We reviewed our experience with the Ross AVR in older children and young adults (10-20 years old) and analyzed mortality, early and late complications, and the need for reoperation and compared it to non-Ross AVR patients of the same age group during the same time period. Between 1993 and 2013, 79 children and young adults, of which 19 were female, between the age of 10 and 20, mean of 16.0 ± 2.7 years, underwent the Ross AVR. Follow-up ranged from 1 month to 20 years with a mean of 6.9 ± 5.8 years. Patients with primary and/or predominant aortic regurgitation and a dilated aortic root and/or ascending aorta at any level were called the "primarily aortic insufficiency (AI) group" (PAIG); 38 (48%) met this criteria. Because we saw some pathologic root enlargement and/or progressive aortic regurgitation in our early Ross experience (1993-2000), we modified our technique and postoperative management in 2000; 51 (65%) of 79 patients underwent the modified technique while 28 underwent our original Ross root replacement technique. The modified technique included reinforcing the aortic valve annulus and sinotubular junction and resecting or replacing the ascending aorta if it was dilated (>30 mm). Twenty-six patients during this same time period and in the same age group underwent the non-Ross AVR with a mean age of 16.5 ± 2 years. Seven of these 26 non-Ross patients were female, and 16 (62%) presented with aortic regurgitation as their primary lesion. The early mortality for the Ross group and the non-Ross group was 0% and 4%, respectively. Late mortality for the Ross group was 2.5% and 0% for the non-Ross group. Of the 28 patients, 14 (50%) receiving the early Ross operation prior to 2000 have required reoperations. Only three (5.9%) of the 51 patients done after 2000 have required surgical reintervention (P <.01). In all, 11 (34%) of our Ross patients operated prior to 2000 and three (6%) after 2000 have required reintervention on their autografts. Patients in the PAIG had zero early and late deaths and a 16% rate of reoperation on the autograft compared to zero early and two late deaths and 20% rate of reoperation in patients not in the PAIG group (P = .266 and .467 respectively). The actuarial survival for the Ross group at 5 (N = 42), 10 (N = 24), and 20 (N = 1) years was 100%, 97%, and 73% respectively. Survival for the non-Ross group at 5 (N = 18), 10 (N = 8), and 20 (N = 1) years was 96%, 96%, and 96%, respectively; this difference in survival was not statistically significant (P =.90). Differences in survival without reoperation for both the groups were not statistically significant (P =.55). When comparing patients who had the newer Ross AVR technique and the non-Ross AVR patients, there was a significantly lower incidence of late aortic stenosis (AS) with a resting gradient greater than 20 mm Hg, 0% versus 53% (P <.001). The Ross AVR is the procedure of choice for adolescents requiring AVR who have a normal pulmonary valve. There was no difference between the outcomes in patients who were in the PAIG group compared to non-PAIG patients. There was no difference in survival without reoperation between the Ross AVR and the non-Ross AVR within the 10- to 20-year age group. The Ross AVR patients had lower incidences of nonoperative complications. The Ross AVR patients had excellent hemodynamic outcomes with a significantly lower incidence of late AS when compared to the non-Ross AVR group. Reports of 15- to 20-year survival for the Ross AVR are encouraging. Long-term follow-up is necessary in all patients with aortic valve disease regardless of the treatment modality.

Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators

Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein's anomaly, Uhl's anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully.

Pulmonary artery xanthogranuloma

A 36-year-old woman with a 6-month history of exertional fatigue was referred for echocardiography. In physical examination, a low-pitched systolic murmur (Grade III/VI) was heard best in the left upper sternal border. Transthoracic echocardiography revealed dilated right cardiac chambers with severe tricuspid regurgitation. Continuous-wave Doppler echocardiography showed an 80 mmHg right ventricular outflow tract gradient with normal pulmonary valve function. Careful examination of great vessels showed …