Ultrasound Pitfalls in a Complex Fetal Cardiac Malformation—Case Report of a New Arteriovenous Central Communication

Cardiac and cardiovascular malformations are of real interest in terms of definition, epidemiology, and means of early diagnosis by imaging. Although ultrasound examination reaches exceptional performance nowadays, unusual pathologies are still exposed to the risk of either incorrect acquired image or misinterpretation by the specialist in a routine scan. Herein, we present a case of a 20-week-old fetus (from an apparently low-risk pregnancy) with complex cardiac and vascular abnormalities, including an arteriovenous malformation along with ventricular septal defect, ductal coarctation of the aorta, aneurysm of a brachiocephalic vein, and dilation of the entire neck and upper mediastinum venous system, and the limitations that were encountered in the process of diagnosis and management of the case.

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Increased Prevalence of Ventricular Septal Defect: Epidemic or Improved Diagnosis

PEDIATRICS ◽

10.1542/peds.83.2.200 ◽

1989 ◽

Vol 83 (2) ◽

pp. 200-203

Author(s):

Gerard R. Martin ◽

Lowell W. Perry ◽

Charlotte Ferencz

Keyword(s):

Ventricular Septal Defect ◽

Septal Defect ◽

Ductus Arteriosus ◽

Coarctation Of The Aorta ◽

Cardiovascular Malformations ◽

Ventricular Septal Defects ◽

Live Births ◽

Septal Defects ◽

Clinical Diagnoses ◽

Control Study

The Baltimore-Washington Infant Study is an ongoing case-control study of congenital cardiovascular malformations in infants in whom the clinical diagnoses have been confirmed by echocardiography, catheterization, surgery, or autopsy. An increase in the prevalence of ventricular septal defects was detected in 1,494 infants with congenital cardiovascular malformations between 1981 and 1984. The prevalence of congenital cardiovascular malformations increased from 3.6 to 4.5 per 1,000 live births (P<.025) and the prevalence of ventricular septal defect increased from 1.0 to 1.6 per 1,000 live births (P< .001). The increase in ventricular septal defects accounted for the total increase in congenital cardiovascular malformations. The prevalence of isolated ventricular septal defect increased from 0.67 to 1.17 per 1,000 live births (P<.001). The prevalence of ventricular septal defect with associated coarctation of the aorta, patent ductus arteriosus, atrial septal defect, and pulmonic stenosis did not change. The prevalence of ventricular septal defect diagnosed by catheterization, surgery, and autopsy did not change; however, defects diagnosed by echocardiography increased from 0.30 to 0.70 per 1,000 live births (P<.001). It is concluded that the reported increase in prevalence of ventricular septal defect is due to improved detection of small, isolated ventricular septal defects and that there is no evidence of an "epidemic."

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Cardiovascular malformations in Omani Arab children with Down's syndrome

Cardiology in the Young ◽

10.1017/s1047951107000078 ◽

2007 ◽

Vol 17 (2) ◽

pp. 166-171 ◽

Cited By ~ 17

Author(s):

Olufemi Jaiyesimi ◽

Vijaymani Baichoo

Keyword(s):

Septal Defect ◽

Down's Syndrome ◽

Coarctation Of The Aorta ◽

Down’S Syndrome ◽

Atrioventricular Septal Defect ◽

Cardiovascular Malformations ◽

Arab Children ◽

Septal Defects ◽

Appropriate Care ◽

Echocardiographic Evaluation

Background and aim: Reports from several countries suggest that the prevalence and spectrum of the congenital cardiac malformations seen in the setting of Down's syndrome vary in different ethnic groups and countries, and at different periods in the same country. Data on Arab children are lacking. Our study aimed to fill that void by ascertaining the pattern in Omani Arabs. Methods: Prospective clinical and echocardiographic evaluation of consecutively recruited Omani children with Down's syndrome. Results: We studied 110 children, aged from one day to 11 years, with a median of 2 months, detecting 76 cardiovascular malformations in 63 (57%) of them. Atrioventricular septal defect, the most prevalent lesion, accounted for 24 (32%) of the 76 defects. Next were atrial septal defects within the oval fossa (29%), patency of the arterial duct (17%), and ventricular septal defect (14%). There were only two cases of Fallot's tetralogy, one each of coarctation of the aorta and right-sided heart, respectively; and none of discordant ventriculo-arterial connections. Conclusion: The prevalence of cardiovascular malformations is high, at 57%, in our studied population of Arab children. Overall, 92% of the malformations are defects which are characterized by a left-to-right shunt, and the potential for pulmonary hypertension. These findings underscore the need for early detection and prompt, appropriate care. To achieve this, if feasible, all babies born with Down's syndrome should have echocardiography in the first month of life.

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PHACES: a neurocutaneous syndrome with anomalies of the aorta and supraaortic vessels

Cardiology in the Young ◽

10.1017/s1047951104002173 ◽

2004 ◽

Vol 14 (2) ◽

pp. 206-209 ◽

Cited By ~ 15

Author(s):

Gerald Wendelin ◽

Erwin Kitzmüller ◽

Ulrike Salzer-Muhar

Keyword(s):

Aortic Arch ◽

Posterior Fossa ◽

Coarctation Of The Aorta ◽

Cardiac Defects ◽

Vascular Abnormalities ◽

Great Vessels ◽

Neurocutaneous Syndrome ◽

The Brain ◽

Supraaortic Vessels ◽

Arterial Anomalies

The acronym PHACES summarizes the most important manifestations of a rare neurocutaneous syndrome. Specifically, “P” accounts for malformation of the brain in the region of the posterior fossa, “H” stands for haemangiomas, “A” is for arterial anomalies, and “C” is for coarctation of the aorta along with cardiac defects, “E” is for abnormalities of the eye, and “S” for clefting of the sternum, and/or a supraumbilical abdominal raphe. Our objective is to introduce the syndrome to paediatric cardiologists. Our patient has stenosis of the aortic arch, multiple malformations of the great vessels arising from the aortic arch, intracranial vascular abnormalities, a sternal malformation with a supraumbilical raphe, and facial haemangiomas. We stress that it is important always to consider the existence of this syndrome in all patients with facial haemangiomas.

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One-Stage Repair of Cardiac and Arch Anomalies without Circulatory Arrest

Asian Cardiovascular and Thoracic Annals ◽

10.1177/021849230301100315 ◽

2003 ◽

Vol 11 (3) ◽

pp. 250-254 ◽

Cited By ~ 1

Author(s):

Kona Samba Murthy ◽

Robert Coelho ◽

Christopher Roy ◽

Snehal Kulkarni ◽

Benjamin Ninan ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Aortic Arch ◽

Septal Defect ◽

Circulatory Arrest ◽

Coarctation Of The Aorta ◽

Innominate Artery ◽

Double Outlet Right Ventricle ◽

Interrupted Aortic Arch ◽

Ischemic Time ◽

Arch Obstruction

Between 1999 and 2002, 23 patients underwent single-stage complete repair of cardiac anomalies and aortic arch obstruction, without circulatory arrest. Median age was 1.2 years. Intracardiac defects included ventricular septal defect in 9, double-outlet right ventricle in 6, d-transposition of the great arteries and ventricular septal defect in 2, subaortic obstruction in 3, and atrial septal defect in 3. Fourteen patients had coarctation of the aorta, 6 had coarctation with hypoplastic aortic arch, and 3 had interrupted aortic arch. Simple techniques were employed such as cannulation of the ascending aorta near the innominate artery and maintaining cerebral and myocardial perfusion. After correction of arch obstruction, intracardiac repair was undertaken. The mean cardiopulmonary bypass time was 169 min, aortic crossclamp time was 51 min, and arch repair took 16 min. There was no operative mortality or neurological deficit. In follow-up of 1–43 months, no patient had residual coarctation. This simplified technique avoids additional procedures, reduces ischemic time, and prevents problems related to circulatory arrest.

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306 A compound analysis of umbilical artery velocimetry in low risk pregnancy

American Journal of Obstetrics and Gynecology ◽

10.1016/0002-9378(91)91046-y ◽

1991 ◽

Vol 164 (1) ◽

pp. 329

Keyword(s):

Umbilical Artery ◽

Low Risk ◽

Risk Pregnancy ◽

Low Risk Pregnancy

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ABNORMAL CAPILLARY MORPHOLOGY IN CONGENITAL HEART DISEASE

PEDIATRICS ◽

10.1542/peds.37.2.316 ◽

1966 ◽

Vol 37 (2) ◽

pp. 316-322

Author(s):

Stella B. Kontras ◽

JoAnn G. Bodenbender

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Septal Defect ◽

Chronic Hypoxia ◽

Normal Children ◽

Vascular Abnormalities ◽

Definitive Surgery ◽

Vascular Compartment

Capillary morphology has been studied in 111 normal children. In 98% of these cases, straight hairpin forms make up over 80% of the capillaries examined. Thirty children with congenital heart disease showed 80% abnormal capillary patterns consisting of dilated, tortuous, and branched loops. These were most marked in cyanotic tetralogy of Fallot and ventricular septal defect with pulmonary hypertension. These cases also were associated with microscopic hemorrhages, increased capillary fragility and actual postoperative hemorrhagic complications. Definitive surgery in two cases, though improving the patient, did not result in changes in the abnormal capillary patterns. It is suggested that the role of the capillary structure in rheology of blood in the microvasculature has largely been ignored and that abnormalities in this vascular compartment may affect perfusion of tissues. The association of abnormalities in coagulation studies, hemorrhage, and thrombosis with congenital heart disease may in part be related to the morphologic vascular abnormalities. The abnormal capillary findings may be due to maturational arrest or dysmaturity of this portion of the cardiovascular system or may be the result of chronic hypoxia.

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CARDIOVASCULAR MALFORMATIONS IN TURNER'S SYNDROME

PEDIATRICS ◽

10.1542/peds.33.6.919 ◽

1964 ◽

Vol 33 (6) ◽

pp. 919-925

Author(s):

Christopher R. Rainier-Pope ◽

Russell D. Cunningham ◽

Alexander S. Nadas ◽

John F. Crigler

Keyword(s):

Cardiovascular Disease ◽

Coarctation Of The Aorta ◽

Turner's Syndrome ◽

Cardiovascular Malformations ◽

Turner’S Syndrome ◽

Pulmonic Stenosis ◽

Cardiovascular Abnormality ◽

Normal Limits

1. Cardiovascular disease associated with Turner's syndrome is more frequent than hitherto reported. In this study, 36 patients were investigated and it was found that in 16 (44%) there was some form of cardiovascular abnormality. 2. Pulmonic stenosis was found to be as frequent as the more commonly reported coarctation of the aorta. 3. Patients with typical Turner's syndrome, who are chromatin negative, may have coarctation of the aorta or pulmonic stenosis. When buccal smears are chromatin positive, stature is within normal limits, or hypertelorism is present, the most likely cardiovascular lesion will be pulmonic stenosis.

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Single-Stage Repair of Coarctation of the Aorta and Ventricular Septal Defect: A Comparison of Surgical Strategies and Resource Utilization

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135117727256 ◽

2017 ◽

Vol 8 (5) ◽

pp. 559-563 ◽

Cited By ~ 4

Author(s):

Connor Callahan ◽

David Saudek ◽

Amanda Shillingford ◽

Sara Creighton ◽

Garick Hill ◽

...

Keyword(s):

Ventricular Septal Defect ◽

Resource Utilization ◽

Septal Defect ◽

Coarctation Of The Aorta ◽

Retrospective Chart Review ◽

Single Stage ◽

Surgical Approaches ◽

Off Pump ◽

Group 2 ◽

Group 1

Background: We sought to compare clinical outcomes and resource utilization for two surgical approaches for single-stage repair of coarctation of the aorta and ventricular septal defect (VSD). Methods: This was a retrospective chart review of 21 consecutive neonates and infants undergoing single-stage repair of coarctation of the aorta and VSD. Group 1 included 13 patients with both arch repair and VSD repair completed via sternotomy. Group 2 included eight patients with off-pump arch repair via left thoracotomy followed by repositioning and VSD repair via sternotomy. Primary clinical outcome was arch reintervention. Secondary outcomes included various measures of resource utilization. Results: Group 1 patients demonstrated younger age at repair (median of 10 days vs 57 days for group 2; P = .05) and lower proximal arch z scores (−4.2 vs −2.3 for group 2; P = .003). Arch reintervention occurred in 0 of 8 patients in group 2 and 1 (7.7%) of 13 patients in group 1 ( P = nonsignificant). Group 2 was associated with lower total charges (US$68,301 vs US$211,723 for group 1; P = .0007), shorter length of stay (8 days vs 23 days for group 1; P = .004), and shorter duration of postoperative mechanical ventilation (0.5 days vs 4.0 days for group 1; P = .0008). Group 2 was also associated with shorter total cardiopulmonary bypass time (86 minutes vs 201 minutes for group 1; P = .0009). Conclusion: Single-stage two-incision repair of coarctation and VSD in appropriately selected patients may be associated with higher value of care. Confirmation of this finding will require further study based on larger numbers of patients.

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