Poor outcome for patients with totally anomalous pulmonary venous connection and functionally single ventricle

2009 ◽  
Vol 19 (6) ◽  
pp. 594-600 ◽  
Author(s):  
Nicodème Sinzobahamvya ◽  
Claudia Arenz ◽  
Julia Reckers ◽  
Joachim Photiadis ◽  
Peter Murin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Right Atrial ◽  
Pulmonary Venous Obstruction ◽  
Actuarial Survival ◽  
Poor Outcome ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

AbstractTotally anomalous pulmonary venous connection, when also associated with a functionally univentricular connection, is known to have a poor outcome. We retrospectively analysed results for 19 patients undergoing surgery for this combination of lesions between 1995 and February 2009.Of the patients, 12 were neonates, with 11 presenting with signs of pulmonary venous obstruction. In 3 patients, a modified Blalock-Taussig shunt had been constructed. The dominant ventricle was of right ventricular morphology in 17 of the 19 patients, and double inlet was present in all bar 1. Pulmonary atresia or stenosis was found in 14 patients, a common atrioventricular junction in 14 patients, and isomerism of the right atrial appendages in 12 patients, respectively. Comprehensive Aristotle scores ranged from 14 to 23.50. The mean was 16.55, with a standard deviation of 2.19. Pulmonary venous rerouting was combined in 6 patients with construction of a modified Blalock-Taussig, in 4 with banding of the pulmonary trunk, in another 4 with a bidirectional Glenn anastomosis, in 3 with creation of a total cavo-pulmonary connection, and in 1 each with enlargement of the right ventricular outflow tract and the Norwood procedure. Of the cohort, 8 patients died early due to pulmonary hypertension, with all patients having Aristotle scores of at least 18 points dying. Among the 11 early survivors, 5 needed mechanical ventilation for over a week, but 5 patients died later, 3 due to pulmonary hypertension and 2 due to infection. Actuarial survival stabilized at 31.6%, with standard error of 10.7%, from one year onwards. Conversion to the Fontan circulation was successful in 5 patients.Our experience confirms that totally anomalous pulmonary venous connection, when associated with the functionally univentricular arrangement, carries one of the worst outcomes in current surgical practice. Use of the Aristotle comprehensive complexity scores effectively discriminates those patients with this condition at particularly high risk.

Rheology of right ventricular outflow tract obstruction: sub-pulmonary membrane developing months after primary intervention to treat pulmonary atresia with intact interventricular septum

2021 ◽  
pp. 1-4
Author(s):  
Baher M. Hanna ◽  
Wesam E. El-Mozy ◽  
Sonia A. El-Saiedi
Keyword(s):  
Right Ventricular ◽  
Interventricular Septum ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Rare Condition ◽  
Surgical Excision ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Ventricular Outflow Tract Obstruction ◽  
The Right

Abstract Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.

Pulmonary atresia with intact ventricular septum: Is it possible to improve survival?

1992 ◽  
Vol 2 (4) ◽  
pp. 391-394 ◽  
Author(s):  
Carlo Vosa ◽  
Paolo Arciprete ◽  
Giuseppe Caianiello ◽  
Gaetano Palma
Keyword(s):  
Right Ventricular ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Shunt Procedure ◽  
The Right ◽  
Overall Mortality

SummaryBetween February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.

Risk factors for augmentation of the flow of blood to the lungs in pulmonary atresia with intact ventricular septum after radiofrequency valvotomy

2005 ◽  
Vol 15 (2) ◽  
pp. 141-147 ◽  
Author(s):  
Mazeni Alwi ◽  
Geetha Kandavello ◽  
Kok-Kuan Choo ◽  
Bilkis A. Aziz ◽  
Hasri Samion ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Arterial Duct ◽  
The Right ◽  
Patent Arterial Duct

Some patients with pulmonary atresia with an intact ventricular septum, mild to moderate right ventricular hypoplasia, and a patent infundibulum remain duct dependent on the flow of blood through the arterial duct despite adequate relief of the obstruction within the right ventricular outflow tract.The objective of our study was to review the risk factors for stenting of the patent arterial duct, or construction of a Blalock-Taussig shunt, in the patients with pulmonary atresia and an intact ventricular septum who remain duct-dependent following radiofrequency valvotomy and dilation of the imperforate pulmonary valve.We reviewed the data from 53 patients seen between November 1995 and December 2001. Of the 47 patients who survived, 6 required stenting of the patent arterial duct, while 4 needed construction of a modified Blalock-Taussig shunt to augment the flow of blood to the lungs at a mean of 7 plus or minus 5.7 days following the initial intervention. The remaining 37 patients required no additional procedures. We compared the findings in these two groups.The mean diameter of the tricuspid valve in the patients requiring early reintervention was 8.5 plus or minus 3.7 millimetres, giving a Z-score of −1.1 plus or minus 1.47, whilst those in the group without early reintervention had values of 10.7 plus or minus 2.2 millimetres, giving a Z-score of −0.58 plus or minus 1.18 (p equal to 0.003). No statistically significant differences were found in right ventricular morphology, McGoon ratio, or residual obstruction across the right ventricular outflow tract after decompression of the right ventricle.The diameter of the tricuspid valve, therefore, appears to be the only factor predicting the need for augmentation of flow of blood to the lungs. As just over one-fifth of our survivors required such augmentation, we hypothesize that stenting of the patent arterial duct may be performed as an integral part of primary transcatheter therapy in patients with pulmonary atresia and intact ventricular septum who have moderate right ventricular hypoplasia and a small tricuspid valve.

Development of right ventricular outflow tract obstruction after double-lung transplantation for primary pulmonary hypertension

1995 ◽  
Vol 5 (3) ◽  
pp. 278-281 ◽  
Author(s):  
Gül Sagin Saylam ◽  
Jane Somerville
Keyword(s):  
Pulmonary Hypertension ◽  
Lung Transplantation ◽  
Right Ventricular ◽  
Primary Pulmonary Hypertension ◽  
Pulmonary Arterial Pressure ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Pulmonary Arterial ◽  
The Right

SummaryWe present a patient with primary pulmonary hypertension who had unusually high pulmonary arterial pressure prior to double-lung transplantation. Obstruction of the right ventricular outflow tract developed after transplantation and progressed over the subsequent two years.

Laser or radiofrequency pulmonary valvotomy in neonates with pulmonary atresia and intact ventricular septum—description of a new method avoiding arterial catheterization

1992 ◽  
Vol 2 (4) ◽  
pp. 387-390 ◽  
Author(s):  
Andrew N. Redington ◽  
Seamus Cullen ◽  
Michael L. Rigby
Keyword(s):  
Right Ventricular ◽  
Pulmonary Valve ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
New Method ◽  
Intact Ventricular Septum ◽  
The Right ◽  
Radiofrequency Perforation ◽  
Blalock Taussig Shunt

SummaryWe describe a new method of transvenous laser or radiofrequency perforation of the pulmonary valve with subsequent balloon pulmonary valvoplasty. The technique obviates the need for arterial catheterisation and considerably shortens the time needed for the procedure. Uncomplicated and successful relief of pulmonary valvar atresia was achieved in both patients attempted, although one ultimately required a Blalock-Taussig shunt because of increasing muscular obstruction of the right ventricular outflow tract.

scholarly journals Right Atrial and Ventricular Adaptation to Chronic Right Ventricular Pressure Overload

Circulation ◽  
2005 ◽  
Vol 112 (9_supplement) ◽  
Author(s):  
Sydney L. Gaynor ◽  
Hersh S. Maniar ◽  
Jeffrey B. Bloch ◽  
Paul Steendijk ◽  
Marc R. Moon
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Right Ventricular ◽  
Right Atrium ◽  
Systolic Function ◽  
Pressure Overload ◽  
Right Atrial ◽  
Chronic Pulmonary Hypertension ◽  
The Right ◽  
Conduit Function

Background— Increased mortality in patients with chronic pulmonary hypertension has been associated with elevated right atrial (RA) pressure. However, little is known about the effects of chronic right ventricular (RV) pressure overload on RA and RV dynamics or the adaptive response of the right atrium to maintain RV filling. Methods and Results— In 7 dogs, RA and RV pressure and volume (conductance catheter) were recorded at baseline and after 3 months of progressive pulmonary artery banding. RA and RV elastance (contractility) and diastolic stiffness were calculated, and RA reservoir and conduit function were quantified as RA inflow with the tricuspid valve closed versus open, respectively. With chronic pulmonary artery banding, systolic RV pressure increased from 34±7 to 70±17 mm Hg ( P <0.001), but cardiac output did not change ( P >0.78). RV elastance and stiffness both increased ( P <0.05), suggesting preserved systolic function but impaired diastolic function. In response, RA contractility improved (elastance increased from 0.28±0.12 to 0.44±0.13 mm Hg/mL; P <0.04), and the atrium became more distensible, as evidenced by increased reservoir function (49±14% versus 72±8%) and decreased conduit function (51±14% versus 28±8%; P <0.002). Conclusions— With chronic RV pressure overload, RV systolic function was preserved, but diastolic function was impaired. To compensate, RA contractility increased, and the atrium became more distensible to maintain filling of the stiffened ventricle. This compensatory response of the right atrium likely plays an important role in preventing clinical failure in chronic pulmonary hypertension.

Congenital right atrial diverticulum, atrial septal defect within the oval fossa, and complex pulmonary valvar obstruction in an infant with chromosome 8(p23.1) deletion

2005 ◽  
Vol 15 (3) ◽  
pp. 306-308 ◽  
Author(s):  
Aungkana Gengsakul ◽  
Suvro S. Sett ◽  
Martin C. K. Hosking
Keyword(s):  
Right Ventricular ◽  
Septal Defect ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Chromosome 8 ◽  
Right Atrial ◽  
Ventricular Compliance ◽  
The Right ◽  
Right Atrial Aneurysm

We describe an unusual right atrial aneurysm, occurring with progressive obstruction of the right ventricular outflow tract and increasing cyanosis, in a nine-month-old boy with chromosome 8(p23.1) deletion. Surgical resection of the diverticulum, and relief of the right ventricular obstruction, was successful, although impairment of right ventricular compliance persists after 19 months follow-up.

Implantation of drug-eluting stents for relief of obstructed infra-cardiac totally anomalous pulmonary venous connection in isomerism of the right atrial appendages

2008 ◽  
Vol 18 (6) ◽  
pp. 628-630 ◽  
Author(s):  
Derek T. H. Wong ◽  
Shi-Joon Yoo ◽  
Kyong-Jin Lee
Keyword(s):  
Septal Defect ◽  
Pulmonary Atresia ◽  
Atrioventricular Septal Defect ◽  
Right Atrial ◽  
Collateral Arteries ◽  
Vertical Vein ◽  
Right Isomerism ◽  
Drug Eluting ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

AbstractWe describe an infant with severe obstruction of infra-cardiac totally anomalous pulmonary venous connection associated with right isomerism, atrioventricular septal defect, pulmonary atresia, and multiple aortopulmonary collateral arteries. Implantation of a stent into the obstructed descending vertical vein provided effective palliation, with a dramatic increase in saturations of oxygen obviating the need for urgent high-risk surgery.

scholarly journals Prognostic relevance of the right ventricular myo-mechanical index (RV-MMI) in patients with precapillary pulmonary hypertension

Open Heart ◽  
2018 ◽  
Vol 5 (2) ◽  
pp. e000903
Author(s):  
Sebastian Greiner ◽  
Ferdinand Goppelt ◽  
Matthias Aurich ◽  
Hugo A Katus ◽  
Derliz Mereles
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Area Under The Curve ◽  
Strain Imaging ◽  
Right Atrial ◽  
Mechanical Index ◽  
Prognostic Information ◽  
Non Invasive ◽  
The Right

ObjectiveThe aim of the prospective New-RV study was to evaluate a parameter for non-invasive quantification of right ventricular (RV) dysfunction in patients with precapillary pulmonary hypertension (PH) that yields prognostic information and is applicable in daily clinical routine.MethodsSixty-five consecutive patients with precapillary PH under guideline conform therapy (43 women, 22 men) underwent clinical assessment, serological testing, as well as a comprehensive transthoracic echocardiography including strain imaging and a detailed assessment of RV haemodynamics.ResultsThe mean follow-up time was 844 days. Sixteen patients died during clinical follow-up. Right ventricular myo-mechanical index (RV-MMI) was calculated by right atrial size, mean RV pressure gradient and strain imaging of the RV free wall, and was measurable in all examinations. RV-MMI was tested for its diagnostic accuracy (sensitivity of 88% and specificity of 73% for an optimal cut-off value of ≤0.31 mm Hg*%; area under the curve=0.85), as well as its predictive value (HR=3.3, 95% CI 1.6 to 7.0, p<0.001), and was compared in detail with established parameters. RV-MMI and N-terminal pro-brain natriuretic peptide (NTproBNP)were independent predictors of survival (HR=2.9, 95% CI 1.4 to 6.2, p=0.006; and HR=2.6, 95% CI 1.5 to 4.6, p=0.001, respectively).ConclusionIn a cohort of patients with precapillary PH, the RV-MMI differentiates the outcome of patients better than other available non-invasive parameters of RV function by preload and afterload adjusted quantification.Trial registration numberNCT01230294.

Echocardiographic characteristics of venous air embolism presenting as reversible pulmonary atresia in a premature neonate

2004 ◽  
Vol 14 (1) ◽  
pp. 102-105 ◽  
Author(s):  
Abhay Divekar ◽  
Rebecca Cases ◽  
Reeni Soni
Keyword(s):  
Mechanical Ventilation ◽  
Right Ventricular ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Air Embolism ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Acute Obstruction ◽  
Venous Air Embolism ◽  
The Right

Air embolism secondary to mechanical ventilation is a rare but well-described complication in premature infants. We describe the echocardiographic appearance of venous air embolism manifesting as acute obstruction of the right ventricular outflow tract in such a premature infant, and review the pathophysiology of acute obstruction of the right ventricular outflow tract secondary to the “air lock” phenomenon. Awareness of the pathophysiology and echocardiographic appearance of venous air embolism may aid in prompt recognition and potential therapy for this lethal complication of mechanical ventilation.

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