Atrioventricular septal defect in fetal life—a clinicopathological correlation

1991 ◽  
Vol 1 (4) ◽  
pp. 334-343 ◽  
Author(s):  
Andrew C. Cook ◽  
Lindsey D. Allan ◽  
Robert H. Anderson ◽  
Gurleen Sharland ◽  
Nuala L. K. Fagg
Keyword(s):  
Septal Defect ◽  
Fetal Echocardiography ◽  
Morphological Characteristics ◽  
Atrioventricular Septal Defect ◽  
Postnatal Life ◽  
Fetal Life ◽  
Clinicopathological Correlation ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
High Incidence

SummaryIn a series of over 7,000 pregnancies referred for fetal echocardiography, atrioventricular septal defects were detected in 103 fetuses, (17% of 594 anomalies). A large proportion (42%) also had isomerism of the atrial appendages, abnormalities in arrangement and morphology of the thoracoabdominal organs, and a high incidence of associated cardiac malformations. When this was associated with abnormalities of rhythm, the fetus often showed signs of intrauterine congestive heart failure; hydrops, pleural effusion and ascites. Those with trisomic karyotypes (predominantly trisomy 21) were associated with usual atrial arrangement and concordant connections of the cardiac segments. Of the 103 fetuses, 53 were examined at autopsy. Extracardiac abnormalities were documented, and detailed morphologic examination and measurements of the heart were made. The ratios of inlet to outlet septal disproportion were not significantly different from those found in postnatal hearts. Again, as in postnatal life, those hearts with common atrioventricular orifice had a greater degree of ‘scooping’ of the ventricular septum compared to those with separate right and left atrioventricular valves. There were no significant differences in these findings in relation to the karyotype of the fetus. There was an increased incidence of ventricular dominance, and also of coarctation or hypoplasia of the aortic arch, in fetal hearts when compared to autopsied hearts obtained in postnatal life. In summary, the distinguishing morphological characteristics of atrioventricular septal defect are present from 16 weeks of gestation.

scholarly journals Complete Atrioventricular Septal Defect and Pulmonary Stenosis Diagnosed in a 49-Year-Old Woman after 10 Uneventful Births

2015 ◽  
Vol 42 (2) ◽  
pp. 166-168 ◽  
Author(s):  
Firat H. Altin ◽  
Okan Yildiz ◽  
Mehmet Karacalilar ◽  
Oyku Tosun ◽  
Ozgen Ilgaz Kocyigit ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Septal Defect ◽  
Medical Literature ◽  
Pulmonary Stenosis ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Complete Atrioventricular Septal Defect ◽  
Complete Atrioventricular ◽  
Spontaneous Delivery

Atrioventricular septal defects constitute 4% of all congenital cardiac malformations. Patients with complete atrioventricular septal defect rarely survive for decades without surgical treatment. Pulmonary stenosis can provide a delicate balance between the pulmonary and systemic circulations and thereby increase longevity. We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. We discuss her successful surgical treatment in terms of the available medical literature.

Echo-morphological correlates in patients with atrioventricular septal defect and common atrioventricular junction

2006 ◽  
Vol 16 (S3) ◽  
pp. 43-51 ◽  
Author(s):  
William T. Mahle ◽  
Girish S. Shirali ◽  
Robert H. Anderson
Keyword(s):  
Septal Defect ◽  
Clinical Presentation ◽  
Three Dimensional ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Canal ◽  
Atrioventricular Junction ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
The Common ◽  
The Right

It is now well recognized that patients fulfilling the diagnostic criterions for the group of hearts usually described as atrioventricular canal malformations, or atrioventricular septal defects, can present with shunting at atrial level, at both atrial and ventricular levels, and on occasion, with shunting only at ventricular level.1,2It is also well recognized that, in most instances, the patients with shunting exclusively at atrial level have separate atrioventricular valvar orifices for the right and left ventricles, this arrangement often described as the “ostium primum” variant of atrial septal defect.3Morphological and echocardiographic studies, however, have shown that, in this variant presumed to represent deficient atrial septation, it is the atrioventricular septal structures, rather than the atrial septum, which are deficient, the phenotypic feature being the presence of a common atrioventricular junction.4,5In this review, we will show how, using modern day echocardiographic techniques, particularly the newly developed potential for three-dimensional display, it is an easy matter to identify the presence or absence of the common atrioventricular junction, and then to demonstrate the various relationships between the valvar leaflets, the septal structures, and the common junction itself which determine the options for clinical presentation within the group.

scholarly journals Double orifice and atrioventricular septal defect: dealing with the zone of apposition†

2019 ◽  
Vol 56 (3) ◽  
pp. 541-548
Author(s):  
Margaux Pontailler ◽  
Moussa Haidar ◽  
Mathilde Méot ◽  
Anne Moreau de Bellaing ◽  
Régis Gaudin ◽  
...  
Keyword(s):  
Valve Replacement ◽  
Septal Defect ◽  
Surgical Repair ◽  
Independent Predictor ◽  
Mechanical Valve ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Double Orifice

AbstractOBJECTIVESA double orifice of the left atrioventricular valve (LAVV) associated with atrioventricular septal defects (AVSD) can significantly complicate surgical repair. This study reports our experience of AVSD repair over 3 decades, with special attention to the zone of apposition (ZoA) of the main orifice, and presents a technique of hemivalve pericardial extension in specific situations.METHODSWe performed a retrospective study from 1987 to 2016 on 1067 patients with AVSD of whom 43 (4%) had a double orifice, plus 2 additional patients who required LAVV pericardial enlargement. Median age at repair was 1.3 years. Mean follow-up was 8.2 years (1 month–32 years).RESULTSAssociated abnormalities of the LAVV subvalvular apparatus were found in 7 patients (5 parachute LAVV and 2 absence of LAVV subvalvular apparatus). ZoA was noted in 4 patients (9%): partially closed in 15 (35%) and completely closed in 24 (56%). Four patients required, either at first repair or secondarily, a hemivalve enlargement using a pericardial patch without closure of the ZoA. The early mortality rate was 7% (n = 3), all before 2000. Two patients had unbalanced ventricles and the third had a single papillary muscle. There were no late deaths. Six patients (14%) required 7 reoperations (3 early and 4 late reoperations) for LAVV regurgitation and/or dysfunction, of whom 4 (9%) required mechanical LAVV replacement (all before 2000). Freedom from late LAVV reoperation was 97% at 1 year, 94% at 5 years and 87% at 10, 20 and 30 years. Unbalanced ventricles (P = 0.045), subvalvular abnormalities (P = 0.0037) and grade >2 LAVV postoperative regurgitation (P = 0.017) were identified as risk factors for LAVV reoperations. Freedom from LAVV mechanical valve replacement was 95% at 1 year, 90% at 5 years and 85% at 10, 20 and 30 years. An anomalous LAVV subvalvular apparatus was identified as a risk factor for mechanical valve replacement (P = 0.010). None of the patients who underwent LAVV pericardial extension had significant LAVV regurgitation at the last follow-up examination.CONCLUSIONSRepair of AVSD and double orifice can be tricky. Preoperative LAVV regurgitation was not identified as an independent predictor of surgical outcome. LAVV hemivalve extension appears to be a useful and effective alternate surgical strategy when the ZoA cannot be closed.

Atrioventricular septal defect associated with type A postaxial polydactyly

2004 ◽  
Vol 14 (2) ◽  
pp. 215-218 ◽  
Author(s):  
Solly E. Levin ◽  
Kathy Vanderdonck
Keyword(s):  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Valve ◽  
Second Child ◽  
Postaxial Polydactyly ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
First Case ◽  
Double Orifice

We report two more patients with the association of postaxial polydactyly and atrioventricular septal defects. Additional cardiac findings, not previously described, were tetralogy of Fallot in the first case, and double orifice right atrioventricular valve in the second child.

What is the optimal time to repair atrioventricular septal defect and common atrioventricular valvar orifice?

2007 ◽  
Vol 17 (4) ◽  
pp. 356-359 ◽  
Author(s):  
Brian E. Kogon ◽  
Hunter Butler ◽  
Michael McConnell ◽  
Traci Leong ◽  
Paul M. Kirshbom ◽  
...  
Keyword(s):  
Intensive Care ◽  
Categorical Data ◽  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Optimal Time ◽  
Ventricular Septal Defects ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Time Required ◽  
Patent Arterial Duct

AbstractObjectiveWith improvements in technology and surgical technique, paediatric cardiologists are challenging surgeons to repair balanced atrioventricular septal defects in smaller patients. Early repair minimizes aggressive medical therapy to prevent heart failure, maintains growth, and limits exposure to elevated pulmonary pressures. We compare the outcomes of repair among different-sized children.MethodsFrom December 2002 to July 2005, 92 patients underwent repair of an atrioventricular septal defect with common atrioventricular valvar orifice and balanced ventricles. We reviewed operative and postoperative data. We excluded patients weighing more than 10 kilograms, but included those who underwent concomitant closure of a patent oval foramen or atrial septal defect, or ligation of a patent arterial duct. Those requiring other concomitant procedures were excluded from the analysis.ResultsThe median weight at repair was 4.9 kilograms, with a range from 2.93 to 7.9 kilograms, and the median age was 5.1 months, with a range from 0.39 to 9.6 months. Operative data included the time required for cardiopulmonary bypass, aortic cross-clamping, and the overall procedure. These times were not significantly affected by decreasing weight. Postoperative continuous data included duration of ventilation and length of intensive care unit and hospital stay. Stay in intensive care (p = 0.006) and hospital (p = 0.007) both increased significantly with decreasing weight. Postoperative categorical data included presence of residual ventricular septal defects, regurgitation across the left atrioventricular valve, and complications. While there was no difference in residual defects (p = 0.166) or valvar regurgitation (p = 0.729), there was a significantly higher presence of complications with decreasing weight (p = 0.0043). There was no mortality, and no persistent heart block requiring placement of a permanent pacemaker.ConclusionsOur data shows that, with the exception of a slightly longer and more complicated postoperative course, early surgery for symptomatic patients with atrioventricular septal defects and common atrioventricular valvar orifice can be undertaken safely and effectively in smaller children with excellent outcomes.

Spectrum and outcome of atrioventricular septal defect in fetal life

2002 ◽  
Vol 12 (1) ◽  
pp. 18-26 ◽  
Author(s):  
Vlasta Fesslova ◽  
Laura Villa ◽  
Simona Nava ◽  
Chiara Boschetto ◽  
Carla Redaelli ◽  
...  
Keyword(s):  
Atrioventricular Block ◽  
Septal Defect ◽  
Complex Form ◽  
Atrioventricular Septal Defect ◽  
Favourable Outcome ◽  
Trisomy 13 ◽  
Chromosomal Anomalies ◽  
Fetal Life ◽  
Atrioventricular Septal Defects ◽  
Left Isomerism

Objectives of the study: to analyse the features and outcomes of different types of atrioventricular septal defects, detected during fetal life, as compared to postnatal data. Material and methods: We analysed retrospectively the data concerning 82 fetuses with atrioventricular septal defect, diagnosed from 19 through 37 weeks gestation with a median of 26 weeks. In 46 cases (56.1%), the diagnosis has been made before the age of 24 weeks. Results: Characteristics of the series – in 44 fetuses the atrioventricular septal defect was not associated with other cardiac anomalies, while 38 fetuses had a more complex form. Chromosomal anomalies were present in 33 of the fetuses (40.2%), more frequently in cases without associated intracardiac defects (56.8%). Trisomy 21 occurred in just over one quarter the series, and in 43.2% of cases without associated defects. In addition, 11% of fetuses had trisomy 18, and one had trisomy 13. Extracardiac anomalies were present in 12 of the fetuses (14.6%), more frequently in cases without associated abnormalities. Of fetuses with more complex defects, 46.4% had hypoplasia of the left ventricle and aorta. Complete atrioventricular block was present in 10 of the fetuses (12.2%), mainly in fetuses with other malformations, and particularly with left isomerism. Recurrence of congenital heart disease was observed in 5 of the fetuses (6.1%). Outcome: In 25 instances (30.5%) the parents opted for termination of pregnancy. Of 57 cases that continued through pregnancy, 9 fetuses died prior to term (15.8%), 32 died postnatally (56.13%) and only 16 fetuses (28.1%) survived. Overall, the mortality was higher in cases with associated malformations, in those with heart failure or those with atrioventricular block. Cardiac surgery was performed in 19 infants, with 5 dying postoperatively, and one late. Conclusions: Our data show a high prevalence of atrioventricular septal defect associated with other malformations when diagnosed during fetal life. This combination is less frequently associated with chromosomal and extracardiac anomalies, but more often with obstructive lesions of the left heart and with atrioventricular block. The association results in a less favourable outcome.

Technique of management of the left atrioventricular valve in the repair of atrioventricular septal defect with a common atrioventricular orifice

1991 ◽  
Vol 1 (4) ◽  
pp. 356-366 ◽  
Author(s):  
Hillel Laks ◽  
Eli R. Capouya ◽  
Jeffrey M. Pearl ◽  
Amir Elami ◽  
Davis C. Drinkwater
Keyword(s):  
Septal Defect ◽  
Surgical Techniques ◽  
Atrioventricular Septal Defect ◽  
Recent Experience ◽  
Atrioventricular Valve ◽  
Early Outcome ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Complete Repair

SummaryDespite improvement in the early outcome for patients undergoing complete repair of atrioventricular septal defects with a common atrioventricular orifice, the incidence of reoperation for failure of the repair of the left atrioventricular valve remains high. The surgical techniques employed in our most recent experience, including routine suture of the septal commissure and liberal use of valvar annuloplasty, were successful in achieving a very satisfactory competence of left atrioventricular valve with a iow incidence of reoperation for failure of the repair.

scholarly journals COMPLETE ATRIOVENTRICULAR SEPTAL DEFECTS DENGAN POLISITEMIA SEKUNDER

2019 ◽  
Vol 8 (2) ◽  
pp. 444
Author(s):  
Bun Yurizali ◽  
AM Hanif
Keyword(s):  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Punctum Maximum ◽  
Complete Atrioventricular

Atrioventricular septal defect (AVSD) adalah kelainan berupa defek pada septum atrioventrikular (AV) di atas atau bawah katup AV, disertai kelainan katup AV; terjadi akibat pertumbuhan yang abnormal dari endokardial cushion pada masa janin. AVSD mewakili 4% sampai 5% bawaan cacat jantung. Dilaporkan seorang pasien laki–laki 20 tahun dengan keluhan sesak nafas saat beraktivitas dan berkurang dengan istirahat, disertai bibir dan kuku jari yang membiru, sakit kepala hilang timbul, muka kemerahan, demam, lemah letih lesu dan dada rasa berdebar-debar. Adanya sianosis, kulit kemerahan, Konjungtiva hiperemis, peningkatan JVP, hepatojugular refluks, bentuk dada abnormal. Bunyi jantung reguler, terdengar bising sistolik di RIC VI, blowing, grade 4/6, punctum maximum di apeks, penjalaran ke Axilla. Bising sistolik di RIC V linea strenalis dektra, grade 4/6, blowing punctum maksimun di RIC V linea sternalis dektra. Pada pemeriksaan laboratorium didapatkan polisitemia. Pada rontgent dada terdapat kardiomegali, dari ekokardiografi adanya CAVSD, hipertensi pulmonal Moderate-severe, Left arch, fungsi RV yang menurun. Pada pasien dilakukan prosedur flebotomi untuk mengurangi kepekatan darah. Pengobatan hipertensi pulmonal pada pasien ini adalah dengan pemberian diuretik dan dorner.

scholarly journals COMPLETE ATRIOVENTRICULAR SEPTAL DEFECTS DENGAN POLISITEMIA SEKUNDER

2019 ◽  
Vol 8 (2) ◽  
pp. 444
Author(s):  
Bun Yurizali ◽  
AM Hanif
Keyword(s):  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Atrioventricular Septal Defects ◽  
Septal Defects ◽  
Punctum Maximum ◽  
Complete Atrioventricular

Atrioventricular septal defect (AVSD) adalah kelainan berupa defek pada septum atrioventrikular (AV) di atas atau bawah katup AV, disertai kelainan katup AV; terjadi akibat pertumbuhan yang abnormal dari endokardial cushion pada masa janin. AVSD mewakili 4% sampai 5% bawaan cacat jantung. Dilaporkan seorang pasien laki–laki 20 tahun dengan keluhan sesak nafas saat beraktivitas dan berkurang dengan istirahat, disertai bibir dan kuku jari yang membiru, sakit kepala hilang timbul, muka kemerahan, demam, lemah letih lesu dan dada rasa berdebar-debar. Adanya sianosis, kulit kemerahan, Konjungtiva hiperemis, peningkatan JVP, hepatojugular refluks, bentuk dada abnormal. Bunyi jantung reguler, terdengar bising sistolik di RIC VI, blowing, grade 4/6, punctum maximum di apeks, penjalaran ke Axilla. Bising sistolik di RIC V linea strenalis dektra, grade 4/6, blowing punctum maksimun di RIC V linea sternalis dektra. Pada pemeriksaan laboratorium didapatkan polisitemia. Pada rontgent dada terdapat kardiomegali, dari ekokardiografi adanya CAVSD, hipertensi pulmonal Moderate-severe, Left arch, fungsi RV yang menurun. Pada pasien dilakukan prosedur flebotomi untuk mengurangi kepekatan darah. Pengobatan hipertensi pulmonal pada pasien ini adalah dengan pemberian diuretik dan dorner.

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