Optimal Z-Score Use in Surgical Decision-Making in Pulmonary Atresia With Intact Ventricular Septum

2017 ◽  
Vol 8 (3) ◽  
pp. 385-388 ◽  
Author(s):  
Mark Nelson Awori ◽  
Nikita P. Mehta ◽  
Frederick O. Mitema ◽  
Naomi Kebba
Keyword(s):  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Data Sets ◽  
Intact Ventricular Septum ◽  
Z Score ◽  
Biventricular Repair ◽  
Tricuspid Valve Annulus ◽  
Z Scores ◽  
Valve Annulus

Objectives: In the surgical treatment of pulmonary atresia with intact ventricular septum, the size of the tricuspid valve annulus (as measured by z-scores) has emerged as a significant factor in deciding which repair to perform. Various tricuspid valve annulus z-scores are reported as “cutoffs” for successful biventricular repair. We aimed to determine whether the use of different z-score data sets contributed to the gross variation in “cutoffs” for successful biventricular repair reported in the literature. Methods: A single search was made of PubMed using the “advanced” setting with the following search terms: pulmonary, atresia, intact, septum, z, and score. The filters “title” and “title/abstract” were used for the first four and last two terms, respectively; the instruction “AND” combined all terms. Articles that identified which z-score data set was used in patients with biventricular repairs were included. Results: From 13 articles, 1,392 patients were studied, 410 (29.5%) of which achieved biventricular repair. Three z-score data sets were quoted; mean tricuspid valve annulus z-scores in biventricular repair patients ranged between −0.53 and −5.1. After correcting for discrepancies between z-score data sets, no study reported a mean tricuspid valve annulus z-score <−2.8 in biventricular repair patients and 83.3% reported mean tricuspid valve annuli z-scores >−1.7. Conclusion: The use of varied tricuspid valve annuli z-score data sets may have contributed to gross variations in reported “cutoffs” for successful biventricular repair. This could lead to inappropriate surgical pathway allocation.

Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum diagnosed during fetal life

2002 ◽  
Vol 12 (5) ◽  
pp. 436-444 ◽  
Author(s):  
George G. S. Sandor ◽  
Andrew C. Cook ◽  
Gurleen K. Sharland ◽  
S. Yen Ho ◽  
James E. Potts ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Fetal Echocardiography ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Fetal Life ◽  
Intact Ventricular Septum ◽  
Z Scores ◽  
Definition Of ◽  
The Right

Objectives: To establish the prevalence of coronary arterial abnormalities in mid-trimester fetuses with pulmonary atresia with intact ventricular septum, and whether their presence correlates with right ventricular morphology. Background: The presence of coronary arterial fistulas significantly alters the surgical options and prognosis for patients with pulmonary atresia with intact ventricular septum. The lesion can reliably be diagnosed using fetal echocardiography, and further definition of the prognosis is important for counselling parents. Methods: We examined the hearts of 39 pathological specimens diagnosed during fetal life, 3 of whom died postnatally. Coronary arterial abnormalities were defined as non-connection of the left or right coronary arteries to the aorta, ostial stenosis, marked tortuosity, dilation, thickening or abnormal myocardial branching. Mild tortuosity, or myocardial bridging, were considered normal. We measured the dimensions of the tricuspid valve along with the inlet and outlet portions of the ventricles. Ebstein's malformation, tricuspid valvar dysplasia, and the presence or absence of the infundibulum, were especially noted. We examined also 12 normal hearts as controls. Results: Coronary arterial abnormalities were found in 14/39 (36%). The dimensions of the right ventricle and tricuspid valves, and the gestational ages of the fetuses, were compared for these 14 with the 25 having no abnormalities using independent t-tests. The gestational ages were similar, 21.9 vs 21.1 weeks. The mean dimensions of the tricuspid valve, median z-scores, and right ventricle were smaller, 2.9 vs 7.2 mm; p < 0.002; −4.46 vs 0.23; p < 0.03; and 6.9 vs 13.7 mm; p < 0.002, for those with coronary arterial abnormalities. Ebstein's malformation, or dysplasia of the tricuspid valve, was present in 4 of 14 with, vs 15 of 25 without, coronary arterial abnormalities. A patent infundibulum was noted in 34 of 39 specimens. Conclusions: Mid-trimester fetuses with pulmonary atresia with intact ventricular septum already exhibit coronary arterial abnormalities, with a prevalence of 36%. The presence of a patent infundibulum confirms that atresia of the pulmonary valve is an acquired process. Coronary arterial abnormalities are seen in 50% of those with hypoplastic right ventricles, but less frequently in the presence of well developed ventricles. This is important information for those involved in counselling parents.

scholarly journals Pulmonary atresia, intact ventricular septum, and Ebstein anomaly of the tricuspid valve

1993 ◽  
Vol 106 (2) ◽  
pp. 255-261 ◽  
Author(s):  
Giovanni Stellin ◽  
Francesco Santini ◽  
Gaetano Thiene ◽  
Uberto Bortolotti ◽  
Luciano Daliento ◽  
...  
Keyword(s):  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Ebstein Anomaly

Echocardiographic factors discriminating biventricular versus univentricular approach in the foetus with borderline left ventricle

2014 ◽  
Vol 25 (5) ◽  
pp. 941-950 ◽  
Author(s):  
David W. Jantzen ◽  
Sarah K. Gelehrter ◽  
Sunkyung Yu ◽  
Janet E. Donohue ◽  
Carlen G. Fifer
Keyword(s):  
Mitral Valve ◽  
Left Ventricle ◽  
Single Ventricle ◽  
Left Ventricular ◽  
Z Score ◽  
Biventricular Repair ◽  
Z Scores ◽  
Valve Annulus ◽  
Single Ventricle Palliation ◽  
Dimension Ratio

AbstractBackground: The term “borderline left ventricle” describes a small left heart that may be inadequate to provide systemic cardiac output and implies the potential need for a single-ventricle palliation. The aim of this study was to identify foetal echocardiographic features that help discriminate which infants will undergo single-ventricle palliation versus biventricular repair to aid in prenatal counselling. Methods: The foetal database at our institution was searched to identify all foetuses with borderline left ventricle, as determined subjectively by a foetal cardiologist, from 2000 to 2011. The foetal images were retrospectively analysed for morphologic and physiologic features to determine which best predicted the postnatal surgical choice. Results: Of 39 foetuses identified with borderline left ventricle, 15 were planned for a univentricular approach, and 24 were planned for a biventricular approach. There were significant differences between the two outcome groups in the Z-scores of the mitral valve annulus, left ventricular end-diastolic dimension, aortic valve annulus, and ascending aorta diameter (p<0.05). With respect to discriminating univentricular outcomes, cut-offs of mitral valve Z-score ⩽−1.9 and tricuspid:mitral valve ratio ⩾1.5 were extremely sensitive (100%), whereas a right:left ventricular end-diastolic dimension ratio ⩾2.1 provided the highest specificity (95.8%). Conclusion: In foetuses with borderline left ventricle, a mitral valve Z-score ⩾−1.9 or a tricuspid:mitral valve ratio ⩽1.5 suggests a high probability of biventricular repair, whereas a right:left ventricular end-diastolic dimension ratio ⩾2.1 confers a likelihood of single-ventricle palliation.

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.

Clinicopathological correlation—pulmonary atresia with imperforate tricuspid valve and aneurysmal dilatation of ascending aorta

1993 ◽  
Vol 3 (3) ◽  
pp. 220-224
Author(s):  
Rafael Hirsch ◽  
Leon Gerlis ◽  
Jane Somerville
Keyword(s):  
Heart Failure ◽  
Atrial Fibrillation ◽  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Ascending Aorta ◽  
Ventricular Septum ◽  
Clinicopathological Correlation ◽  
Intact Ventricular Septum ◽  
Aneurysmal Dilatation ◽  
Unusual Form

AbstractA patient with the rare combination of an unusual form of pulmonary atresia with intact ventricular septum and a Marfan-like disease is presented. She was extremely cyanosed in childhood but improved after construction of a left peripheral shunt at the age of nine years. She deteriorated gradually after the onset of atrial fibrillation at the age of 33, the subsequent course being complicated by a stroke and, finally, by intractable heart failure and death at the age of 39.

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