Dysplastic Valve Research Articles

Craniosynostosis surgery in an infant with a complex cyanotic cardiac defect

We report on a case where craniosynostosis surgery for a left-sided coronal synostosis was performed successfully on an 11-month old infant with a hypoplastic left ventricle with a dysplastic mitral valve, double outlet right ventricle, transposition of the great arteries, atrial septal defect, multiple ventricular septal defects, and surgically applied pulmonary banding. Craniosynostosis surgery is considered high-risk surgery, because of possible sudden and extensive blood loss, and is usually performed in cardiopulmonary healthy children. Children with congenital heart disease undergoing noncardiac surgery have an increased risk of perioperative morbidity and cardiac arrest. Patients with hypoplastic left heart syndrome are a high-risk population when undergoing noncardiac surgery, in all stages of palliation. This infant would be undergoing a partial cavo-pulmonary connection (PCPC) within a few months. With a PCPC, drainage of cranial vessels is dependent on passive flow via the superior caval vein directly into the pulmonary artery. Consequently, this could lead to an increased blood loss during craniosynostosis surgery. Therefore, it was decided to perform the craniosynostosis surgery first, before establishing a PCPC. When a child with CHD presents for high-risk noncardiac surgery, future cardiac procedures and physiology also have to be taken into account. A multidisciplinary approach, involving pediatric cardiologists and pediatric anesthesiologists, is essential in making this decision.

Double‐Outlet Right Atrium Associated with Hypoplastic Right Heart Structures and Aortic Stenosis: Echocardiographic Features

Mini‐AbstractDouble‐outlet right atrium, also known as straddling tricuspid valve without a ventricular septal defect, is an extremely rare congenital cardiac anomaly, in which the tricuspid valve has 2 ostia. We describe an infant with this finding in association with hypoplastic right heart structures and a dysplastic aortic valve.

10.5937/mckg48-6133 = Congenital mitral valve dysplasia in a female child: Infective endocarditis or heritable disorder of connective tissue?

Introduction. Dysplastic or myxomatous mitral valve is characterized by myxomatous degeneration of collagen and elastin which causes excess growth of mitral valves. In due course, some pathological characteristics may develop, such as mitral valves prolapse with considerable regurgitation, while surface abnormalities of such valves might predispose thromboembolic complications or infective endocarditis. Case report. Three years and seven months old female child was suspected of having infective endocarditis based on the medical history, clinical signs and performed investigations. According to the Duke criteria, she had: 1 major sign - positive echocardiographic finding on the heart (existence of 'verrucae' on mitral valve apparatus) and 3 minor signs - prolonged fever, one positive blood culture and arthralgia. After the applied antibiotic therapy, the control echocardiographic finding was unchanged, laboratory analyses were within normal range and the general condition of the child was good. Therefore, we considered it as myxomatous degeneration of mitral valve apparatus which is very similar to verrucous changes. Conclusion. Based on similar phenotype and echocardiographic characteristics of the little girl and her father we believe that the case in question was congenital mitral valve dysplasia with possible heritable disorder of connective tissue.

Early Postoperative Outcomes Following Surgical Repair of Complete Atrioventricular Septal Defects

To evaluate the impact of Down syndrome on the early postoperative outcomes of children undergoing complete atrioventricular septal defect repair. Retrospective cohort study. Single tertiary pediatric cardiac center. All children admitted to PICU following biventricular surgical repair of complete atrioventricular septal defect from January 2004 to December 2009. None. A total of 107 children, 67 with Down syndrome, were included. Children with Down syndrome were operated earlier: 4 months (interquartile range, 3.5-6.6) versus 5.7 months (3-8.4) for Down syndrome and non-Down syndrome groups, respectively (p < 0.01). There was no early postoperative mortality. There was no significant difference in the prevalence of dysplastic atrioventricular valve between the two groups. Two children (2.9%) from Down syndrome and three children (7.5%) from non-Down syndrome group required early reoperation (p = 0.3). Junctional ectopic tachycardia was the most common arrhythmia, and the prevalence of junctional ectopic tachycardia was similar between the two groups (9% and 10% in Down syndrome and non-Down syndrome, respectively, p = 1). One patient from each group required insertion of permanent pacemaker for complete heart block. Children with Down syndrome had significantly higher prevalence of noncardiac complications, that is, pneumothorax, pleural effusions, and infections (p < 0.01), than children without Down syndrome. There was a trend for longer duration of mechanical ventilation in children with Down syndrome (41 hr [20-61 hr] vs 27.5 hr [15-62 hr], p = 0.2). However, there was no difference in duration of PICU stay between the two groups (2 d [1.3-3 d] vs 2 d [1-3 d], p = 0.9, respectively). In our study, we found no difference in the prevalence of atrioventricular valve dysplasia between children with and without Down syndrome undergoing complete atrioventricular septal defect repair. This finding contrasts with previously published data, and further confirmatory studies are required. Although clinical outcomes were similar, children with Down syndrome had a significantly higher prevalence of noncardiac complications in the early postoperative period than children without Down syndrome.

Cardiac MRI underlines the role of BNP and hematologic parameters as heart failure markers in patients with Ebstein's anomaly of the tricuspid valve

Background Ebstein’s anomaly of the tricuspid valve involves a congenitally displaced and dysplastic tricuspid valve (TV). The displacement of the TV towards the apex results in an enlarged right atrium (RA) with an atrialzied portion of the right ventricle (aRV) and an enlarged functional right ventricle (fRV). Brain natriuretic peptide (BNP) is a heart failure marker. Its role in Ebstein’s anomaly has not been evaluated in conjunction with cardiac MRI (CMR) parameters. Hematologic parameters (hemoglobin (Hb), hematocrite (Hct)) are upregulated in states of pulmonary hypo perfusion as in congenital cyanotic heart disease. Intermittent Pulmonary hypo perfusion may be a an indicator of right heart failure in patients with Ebstein’s anomaly, but has not been studied so far. The aim of the present study was to correlate CMR functional parameters and severity of disease with BNP and hematologic parameters in patients with Ebstein’ sa nomaly. Methods 26 patients with non-corrected Ebstein’ sa nomaly were studied prospectively. Laboratory parameters (BNP, Hb, Hct), CMR data (RA, aRV, fRV, left atrial (LA) and left ventricular (LV) volumes and functional parameters like ejection fraction (EF), stroke volume (SV) and others) as well as exercise capacity (maximal oxygen uptake (VO2 max.), CO2 equivalent (VE/VCO2), maximum heart rate (max. HR) using a cycle ergometer) were all measured on the same day for an individual patient. Results

Fatal Infant Myocardial Infarction Caused by Ball-IN-Valve Mechanism from a Dysplastic Aortic Valve

BackgroundAcute myocardial infarction (AMI) can occur in infants. Early identification of AMI can allow for specific therapies aimed at improving patient outcomes. ObjectiveThis article describes a case of an infant with AMI caused by a previously unreported mechanism and reviews diagnostic and therapeutic strategies for dealing with this rare disease. Case ReportWe present a case of a 5-week-old infant with a fatal AMI due to a pedunculated nodule from a dysplastic aortic valve leaflet creating a ball-in-valve mechanism and occluding the left main coronary artery. ConclusionAMI is an important diagnostic consideration in any infant presenting with signs of unexplained cardiogenic shock. Practitioners who care for children should be aware of diagnostic and therapeutic strategies for AMI in infants.

Smart Nature. Aortico‐Left Ventricular Tunnel Bypassing Congenital Critical Aortic Stenosis

Mini‐AbstractThe presented case illustrates a neonate with aortico‐left ventricular tunnel (ALVT) and critical aortic stenosis (AS), who successfully underwent Ross procedure. Case: A neonate presented with critical AS due to a dysplastic aortic valve and an ALVT, consisting of an aneurysmatic, extracardiac component and an aortic insertion of the tunnel just above the right coronary sinus. The child underwent successful Ross operation and resection of the ALVT on 9th day of life. Follow‐up showed normal left ventricular function and no AS. Conclusion: This is the first reported case of ALVT and critical AS that underwent successful neonatal Ross procedure.

First report of a de novo 18q11.2 microdeletion including GATA6 associated with complex congenital heart disease and renal abnormalities

Deletions of the long arm of chromosome 18 have been previously reported in many patients. Most cases involve the more distal regions of the long arm (18q21.1->qter). However, proximal interstitial deletions involving 18q11.2 are extremely rare. Here we report on a 14-month-old female with a 4.7 Mb (19,667,062-24,401,876 hg19) de novo interstitial deletion within chromosomal band 18q11.2, which includes GATA6 and 24 other RefSeq genes. The clinical features of our patient include complex congenital heart defects, a double outlet right ventricle, a subaortic ventricular septal defect, D-malposed great arteries, an atrial septal defect, a dysplastic aortic valve and patent ductus arteriosus. In addition, she had renal anomalies-a duplicated collecting system on the left and mild right hydronephrosis. These heart and renal defects are not reported in other patients with 18q proximal interstitial deletions. Heterozygous point mutations in GATA6, encoding for a zinc finger transcription factor, have been shown to cause congenital heart defects. Given the well-established biological role of GATA6 in cardiac development, a deletion of GATA6 is very likely responsible for our patient's complex congenital heart defects. This is the smallest and most proximal 18q11.2 deletion involving GATA6 that is associated with complex congenital heart disease and renal anomalies.

Aortic Valve Disease in Pulmonary Atresia and Major Aortopulmonary Collaterals

Aortic valve disease in association with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (PA/VSD/MAPCAs) is an extremely rare combination of congenital heart defects. The presence of aortic stenosis or insufficiency or both imposes an additional physiologic burden that complicates the management of PA/VSD/MAPCAs. This report summarizes our experience with 7 patients who underwent surgical repair of this rare combination of defects. This was a retrospective review of patients who had both aortic valve disease and PA/VSD/MAPCAs. Four patients had predominantly aortic stenosis, 2 patients had aortic insufficiency, and 1 had mixed stenosis and insufficiency. Anatomically, this corresponded to a dysplastic trileaflet valve in 4, bicuspid valve in 2, and quadricuspid valve in 1. Four of the 7 patients underwent aortic valve surgery at the time of their first operation for PA/VSD/MAPCAs. The remaining 3 patients had aortic valve surgery at subsequent operations. All 7 patients are alive, with a median duration of follow-up of 3 years. One patient has required aortic valve replacement 2 years after repair of an insufficient bicuspid valve. This manuscript summarizes our experience with 7 patients who had the rare combination of aortic valve disease and PA/VSD/MAPCAs. Although aortic valve disease has been reported for other conotruncal defects, we believe this is the first report of its presence in association with PA/VSD/MAPCAs.

Giant Right Atrium Due to Congenital Dysplastic Tricuspid Valve in an Elderly Female Patient

Isolated tricuspid valve disease is rare. We report the case of a 65-year-old female patient who presented with right heart failure. Initial echocardiographic evaluation revealed a giant right atrium (RA) with severe tricuspid inflow obstruction with regurgitation. A comprehensive two- and three-dimensional echocardiographic evaluation revealed a dysplastic tricuspid valve resulting in the inflow obstruction and regurgitation. Rheumatic and carcinoid etiologies were ruled out by relevant tests. It is very rare for dysplastic tricuspid valve to present in late adulthood. The classic echocardiographic findings are discussed followed by a brief review of the literature.

Cardiac anomalies in Axenfeld–Rieger syndrome due to a novel FOXC1 mutation

Axenfeld-Rieger syndrome (ARS) is an autosomal dominant condition characterized by ophthalmologic anterior segment abnormalities and extraocular findings including dental anomalies and redundant periumbilical skin. Intragenic mutations in the homeobox gene PITX2 or the transcription factor encoding FOXC1 were identified, and genomic rearrangements encompassing either gene also cause ARS. A molecular etiology is identified in 40-60%. Extraocular anomalies occur more often with intragenic PITX2 than FOXC1 mutations. We report on a patient with infantile glaucoma presenting at age 21 months with congestive heart failure due to a dysplastic arcade mitral valve necessitating valve replacement, and mildly hypoplastic left ventricular outflow tract and aortic arch. Family history included early onset glaucoma in four relatives; congenital hip dysplasia requiring surgery in three; and an atrial septal defect in the affected maternal grandmother. Despite the absence of dental or umbilical abnormalities, anterior chamber abnormalities consistent with ARS were present in affected individuals. Molecular testing revealed a novel FOXC1 mutation (c.508C>T; p.Arg170Trp) in the proband and his affected mother; other family members were unavailable. A literature review revealed four reports of congenital heart disease associated with intragenic FOXC1 mutations, and none with intragenic PITX2 mutations. Previously, mouse studies showed Foxc1 (Mf1) expression in the developing valves and atrial septum, supporting a causal relationship of FOXC1 mutations for valvar anomalies and ASD. Hip dysplasia in three family members suggests a role for FOXC1 in the femoral head dysplasia of de Hauwere syndrome with 6p25 deletions. Further reports of clinical and molecular diagnoses will clarify genotype-phenotype correlation.

Double-Outlet Right Ventricle Without Interventricular Communication: An Unusual and Challenging Problem

A ventriculoseptal defect (VSD) usually is present in patients with a double-outlet right ventricle. This report describes a case of double-outlet right ventricle with a completely shrouded inlet VSD and no interventricular shunting. This was associated with a severely regurgitant dysplastic mitral valve. The anatomy and clinical management of the patient is discussed together with an overview of the existing literature.

Repair of Congenitally Dysplastic Aortic Valve by Bicuspidization: Midterm Results

Repair of congenital aortic valve (CAV) lesions may be achieved by creation of either tricuspid or bicuspid valve morphology. To define feasibility and outcome of CAV repair by bicuspidization a 10-year experience was reviewed. Between January 2002 and December 2011, 147 consecutive patients underwent operation for CAV insufficiency; 58 had valve or root repair (group 1) and 89 had valve or root replacement (group 2). Patients having repair were younger (42.9 vs 51.3 years, p=0.001), with lesser prevalence of severe insufficiency (72% vs 90%, p=0.002). In patients having repair, morphology of CAV was bicuspid in 51, monocuspid in 4, and quadricuspid in 3, whereas in the replacement group it was bicuspid in 87 and quadricuspid in 2 (p=0.04). Surgery consisted of an isolated aortic valve procedure in 20 versus 45 patients, associated with aortic root or ascending aortic repair in 38 versus 44 patients, in group 1 versus 2 (p=0.04). There were no hospital and 3 late deaths during a mean follow-up of 3.8±2.5 years (range 0.2 to 10.0). Eight-year survival (89%±10% vs 97%±2% [p=0.7]), freedom from valve-related events (84%±10% vs 89%±4% [p=0.8]), and freedom from aortic valve reoperation (95%±3% vs 93%±3% [p=0.6]) were comparable. Risk factors for reoperation at univariate analysis were isolated valve surgery (p=0.001), Ross operation (p=0.001), and endocarditis (p=0.002). Follow-up echocardiography of repair patients showed mild or less aortic insufficiency in 51 (88%) and mild or less stenosis in 57 (98%). Valve repair by preservation or creation of bicuspid morphology is feasible in almost half of all comers with CAV insufficiency, with satisfactory and stable midterm functional outcome. Rates of valve-related adverse events and reoperation are similar to those of patients having replacement.

Specific association of missense mutations in CRELD1 with cardiac atrioventricular septal defects in heterotaxy syndrome

Specific association of missense mutations in <i>CRELD1</i> with cardiac atrioventricular septal defects in heterotaxy syndrome

An unusual fistula closed by percutaneous coil embolisation

A 25-year-old male patient was admitted for assessment of increasing shortness of breath and decreased transcutaneous oxygen saturation (91 %). He had a complex congenital heart defect, involving a dysplastic monocusp tricuspid valve and pulmonary valve atresia. The latter was palliated shortly after birth by a Brock procedure (entailing infundibular resection and pulmonary valvotomy). An atrial septal defect was surgically closed when he was 6 years old and a homograft was inserted in the right ventricular outflow tract at the age of 15. Replacement by a second homograft at the age of 23 was necessary together with tricuspid valvoplasty because of severe insufficiency of both valves. Apart from moderate pulmonary homograft stenosis (PHS) and re-occurrence of moderate to severe tricuspid insufficiency (TI) resulting in right heart failure, a right-left shunt was detected on transoesophageal echocardiography during intravenous agitated saline bubble-contrast injection, although the repaired atrial septum was intact. Invasive evaluation confirmed moderate to severe TI and a peak-to-peak gradient of 40 mmHg over the homograft. Right atrial pressures were above 20 mmHg. Contrast injection in the superior vena cava showed a very tortuous fistulous connection originating from the left axillary vein and draining towards the left atrium (Fig. 1, panel A and B). It was decided to close the fistula percutaneously and 4 coils (3 x IMWCE-3-PDA3 and 1 x IMWCE-3-PDA5, COOK MEDICAL, Bloomington, USA) were placed in the fistula, effectively occluding the vessel (panel C). The transcutaneous oxygen saturation increased slightly. Further management regarding the TI and PHS will be discussed with one option being percutaneous placement of a pulmonary valve (Medtronic, Minneapolis, USA), possibly in combination with another transcatheter valve prosthesis in tricuspid position to lower the right heart filling pressures. Fig. 1 a Contrast injection in the superior vena cava (SVC) showing a fistula (arrow) originating from the left axillary vein (AV). The right atrium (RA) is grossly enlarged (LIV left innominate vein). b Selective contrast injection in the fistula (arrow) reveals ...

Mid-term results of bidirectional cavopulmonary anastomosis and hemi-Mustard procedure in anatomical correction of congenitally corrected transposition of the great arteries

The Senning or Mustard procedure combined with the arterial switch operation (ASO) (± VSD and no left ventricular (LV) outflow tract obstruction) or the Rastelli operation (VSD and LV outflow tract obstruction) has become the preferred strategy over conventional repair as it is thought to prevent long-term dysfunction of the right ventricle (RV). More recently, hemi-Mustard rerouting of blood from the inferior vena cava to the RV in combination with bidirectional cavopulmonary anastomosis (BCPA) has been adopted by some centres for potential benefits over the classic atrial switch procedure. The aim of this study was to analyse our experience with hemi-Mustard and BCPA as part of an anatomical repair of congenitally corrected transposition of the great arteries (CCTGA) in selected patients. Between 2004 and 2011, eight patients underwent hemi-Mustard/BCPA with the Rastelli operation (n = 6) or ASO (n = 2). The median age was 2.9 (range: 1.2-9.1) years. Positional anomalies were present in 75% of the patients. Both patients with ASO had dysplastic and insufficient tricuspid valves. In the Rastelli group, four patients had previously received shunts followed by BCPA in one patient. In the ASO group, both patients underwent pulmonary artery banding initially. There was one in-hospital death and no late mortality. Two patients received a pacemaker. One patient from the Rastelli group required conduit change 6 years later. At the mean follow-up of 4.5 years, six and one patients are in NYHA classes I and II, respectively; six patients showed good biventricular function, while one had LV dysfunction. Systemic venous obstruction and sinus node dysfunction were not observed, and BCPA was functioning well in all patients. Hemi-Mustard/BCPA is useful in anatomical repair of CCTGA in selected patients. When compared with the classic atrial switch operation, it is technically easier which makes it especially helpful in atrio-apical discordance; it unloads an RV with limited size or function, and avoids complications related to the upper limb of the classic atrial switch procedure. Mid-term results of this approach are favourable. Further follow-up is needed to prove long-term benefits.

Long-Term Results of Reoperation for Left Atrioventricular Valve Regurgitation After Correction of Atrioventricular Septal Defects

Long-term results of reoperation for left atrioventricular valve regurgitation (LAVVR) after previous correction of atrioventricular septal defect (AVSD) are scarce. We evaluated long-term outcome of reoperation for LAVVR and identified risk factors for reoperation. Between December 1976 and July 2006, 45 of 312 patients with correction of different AVSDs underwent reoperation for LAVVR. The cohort of 267 patients who did not need reoperation for LAVVR allowed for the identification of risk factors for reoperation and evaluation of overall survival after primary AVSD repair in a competing risk scenario. Clinical data were obtained by retrospective review. The left atrioventricular valve (LAVV) was repaired in 31 patients (68.9%) and replaced in 14 (31.1%). There were 3 in-hospital deaths (6.7%) and 2 late deaths (4.4%). Estimated overall survival was 88.1% at 15 years after the reoperation, and estimated incidence of death after reoperation in the total patient cohort was 2% at 15 years after the primary AVSD repair. Overall survival was significantly higher after LAVV repair than after replacement (p=0.010). Ten patients with LAVV repair required a second reoperation for LAVVR. At follow-up, survivors were in New York Heart Association functional class I (n=36) or II (n=4). Independent risk factors for first reoperation for LAVVR were associated cardiovascular anomalies (p<0.001), LAVV dysplasia (p<0.001), and nonclosure of the cleft (p=0.027). After previous correction of AVSD, LAVVR can usually be corrected by valve repair. A very dysplastic valve may necessitate replacement. Overall survival is higher after repair than after replacement. In general, overall survival of patients reoperated on for LAVVR is favorable. The overall mortality rate after primary repair of AVSD is explained only for a small part by mortality after reoperation for LAVVR.

Cutting balloon for the treatment of resistant pulmonic valve stenosis

Background: Congenital pulmonic valve stenosis comprises 7.5–9% of congenital heart defects. The treatment of choice is percutaneous balloon valvuloplasty; however, a small percentage of patients have thick and flexible valve leaflets (dysplastic valves) that require surgical valvotomy. We aim to report our experience using a cutting balloon and repeated balloon dilation as a last attempt before surgical referral. Methods: Between June 2005 and November 2010, we treated 50 patients with isolated pulmonic valve stenosis. Five patients (10%) had thick and dysplastic pulmonic valve leaflets that were resistant to repeated balloon dilation attempts. We attempted to create a tear in the dysplastic pulmonic valve by performing pulmonic valvotomy using a cutting balloon (Boston Scientific, Natick, MA, USA), which was advanced through a long sheath, followed by repeated standard balloon dilation. Results: There was no change in the peak systolic gradient at catheterization after conventional pulmonic valve balloon dilation. However, a reduction of 37% was observed, during catheterization, after cutting balloon dilation and additional standard balloon dilation (p = 0.09). Echocardiographic follow-up showed a 23% decrease of the maximum instantaneous gradient early after cutting balloon dilation of the pulmonic valve (from 75 ± 15 to 58 ± 4 mmHg, p = 0.05). There was no restenosis of the valve (50 ± 13 mmHg) at long-term 5.5 years ± 9 months follow-up (range 4.5–6 years) and no complications. Conclusion: The use of cutting balloon is a possible option for the treatment of resistant congenital pulmonic valve stenosis before referral for surgical valvotomy. A larger controlled trial is needed to assess the effectiveness and safety of the procedure and validate our experience in this small series.

Persistent embryonic right venous valve giving a cor triatriatum dexter appearance in a cyanotic neonate

Embryonic right venous valve may persist after birth as a membrane seen inside the right atrium and give a cor triatriatum dexter appearance in echographic imaging. The finding is usually asymptomatic, but may rarely cause cyanosis when flow from the inferior vena cava is redirected into the left atrium and be misdiagnosed with a tricuspid valve anomaly. We report the case of a cyanotic newborn who underwent a successful resection of a cor triatriatum dexter membrane.

Invited Commentary

Alghamdi and associates [1Alghamdi A.A. Yanagawa B. Singh S.K. Horton A. Al-Radi O.O. Caldarone C.A. Balancing stenosis and regurgitation during mitral valve surgery in pediatric patients.Ann Thorac Surg. 2011; 92: 680-684Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar] have highlighted the delicate balance that can be achieved between mitral stenosis and mitral regurgitation when repairing the mitral valve in children in order to achieve a more durable outcome and avoid mitral valve replacement. They show that greater than mild to moderate mitral regurgitation is poorly tolerated and an independent predictor of reoperation or death. The reduction of mitral regurgitation to less than moderate to mild by the creation of mild mitral stenosis makes for a better long-term repair. This approach of avoiding mitral valve replacement at the expense of a less-than-perfect repair, but one that is well tolerated with balanced regurgitation and stenosis, does allow for growth of the patient and removes the risks of anticoagulation with mitral valve replacement. Deferring mitral valve replacement to the older and larger patient allows a bigger valve to be placed. This article deals with patients of a median age of 4 years, with dysplastic valve morphology rather than the complex morphology seen in infancy, such as parachute mitral valve and where multiple obstructive lesions are present on the left side of the heart (Shone syndrome). Patients in the first few months and year of life are almost certainly more challenging than the cohort in article by Alghamdi and colleagues. However, the approach of achieving balanced mitral stenosis and mitral regurgitation, and a repaired valve, is certainly applicable to this younger, more difficult group. Long-term evaluation outcome for these patients should include the ability of the patient to thrive and develop and the lack of development of pulmonary hypertension. The mitral valve hemodynamics were assessed on the operating table under anaesthetic and probably underestimates the degree and severity of mitral stenosis and regurgitation. However, because this assessment mode was uniformly applied to all patients, it would seem to be acceptable. Mitral valve repair in young patients, particularly in the first few months of life, is difficult and challenging. The underlying morphology is complex, and individual variation in morphology from patient to patient makes each case a separate challenge. Although this report is in older children, the concept that a balanced repair that leaves mild to moderate valve regurgitation together with mild mitral stenosis to compensate for what otherwise would be severe mitral regurgitation, with a very bad outcome, seems eminently sensible. Balancing Stenosis and Regurgitation During Mitral Valve Surgery in Pediatric PatientsThe Annals of Thoracic SurgeryVol. 92Issue 2PreviewThe objectives of this study were to evaluate the composite outcome of reoperation or death for mitral valve repair and replacement and to determine the relative importance of regurgitation and stenosis during mitral valve repair to guide intraoperative decision making. Full-Text PDF