ONE OF THE EARLIEST CLINICAL AND POSTMORTEM DESCRIPTIONS OF CYANOTIC CONGENITAL HEART DISEASE REPORTED IN THE UNITED STATES

PEDIATRICS ◽  
1969 ◽  
Vol 43 (4) ◽  
pp. 577-577
Author(s):  
T. E. C.

The first volume of the New England Journal of Medicine and Surgery, which appeared in 1812, contained this succinct description of congenital cyanotic heart disease in a young girl: S.R. when born was for a considerable time supposed to be dead-did not cry, or evince any living actions. The lungs were artificially inflated for several minutes and life at length appeared, but very feebly.-A livid countenance, with frequent syncope took place.-With great maternal care the infant was kept alive, and as she grew became remarkably sprightly and active. When two years old (she) was unusually intelligent and fond of exercise.-As she advanced in age her fondness for violent exercise in playing often exposed her danger, as these efforts never failed to produce syncope and a kind of convulsion, laughing, crying, any emotion of mind, also brought on the syncope, from which after falling into a horizontal position, she generally soon recovered.-Her countenance, all times blueish and livid, was in these fits extremely extremely so. Her nails were always of the colour of litmus, or perhaps a little nearer to violet. After death the thorax was examined-it was of an unusual shape, being more cylindrical than common, and the lungs having less the form of a cloven hoof, when inflated, than they usually assume.-The heart was very small. In place of a right auricle was observed a small appendage like the edge of that portion of the heart, not capable of containing more than one fourth its usual contents.

2018 ◽  
Vol 17 (05) ◽  
pp. 161-167
Author(s):  
Juan Leon-Wyss ◽  
Cynthia Rosario ◽  
Janet Toribio ◽  
Herwin Speckter ◽  
Bernd Foerster ◽  
...  

AbstractThis article examines the relation between oxygen saturation and T2 star time in cyanotic congenital heart disease and its correlation to cerebral gray and white matter alterations. Magnetic resonance imaging was performed in 25 patients (mean age: 52.2 months) and 32 controls. Gray and white matter volumes, as well as fractional anisotropy and longitudinal diffusivity, were significantly reduced in patients. The reduction longitudinal diffusivity correlated to oxygen saturation and T2 star time of gray matter (p < 0.05). This diffusion tensor imaging (DTI) parameter was most affected in cyanotic congenital heart disease and because is the only parameter showing significant correlation to reduced oxygenation, it should be included more often in the follow-up of these patients over time.


2012 ◽  
Vol 2012 ◽  
pp. 1-3
Author(s):  
Matteo Boattini ◽  
André Almeida ◽  
Rita Barata Moura ◽  
Miguel Toscano Rico

We report the case of a 50-year-old HIV-positive woman with a congenital cyanotic heart disease who developed left axillary, subclavian, and brachiocephalic vein thrombosis and left lower aortopulmonary collateral arterial thrombosis, presenting as left hemibody swelling. We also briefly overview the literature regarding upper extremity deep vein thrombosis (UEDVT). Given the absence of other risk factors, it was our firm believe that our patient's UEDVT was due to a hypercoagulable state associated with congestive heart failure (CHF) and HIV infection.


Author(s):  
Asish Banerjee ◽  
Meenakshi Mitra

Background: Children with Congenital Heart Disease have associated structural neurological    abnormalities and those surviving infancy are even subjected to various environmental factors   which might contribute to neurological abnormalities. Hence the objective is to study the neurological abnormalities of children with Congenital Heart Disease.Methods: A retrospective cross-sectional observation study was conducted over the period of 3 years (June 2016-May 2019). Data were collected from medical records department of 121 children of 2-12 years of age diagnosed with congenital heart disease. Neurological examination findings and neuro imaging and EEG findings where relevant    were noted. To assess the association of adverse neurological outcome and congenital heart disease, data analysis was        performed using Fisher's exact test.Results: 38% children of 2-12 years of age with congenital heart disease had adverse neurological profile. Neurological abnormalities were significantly associated with cyanotic heart disease (p value 0.0001). Statistically significant association were found between congenital cyanotic heart disease and seizure (p value=0.04), hemi paresis (p value=0.039), brain abscess (p value=0.012) and coma (p=0.01).Conclusions: Congenital cyanotic heart disease has significant associations with neurological abnormalities in children. These results demand attention for the   neurological health of the largely uncorrected cardiac disease in Indian children


PEDIATRICS ◽  
1968 ◽  
Vol 42 (4) ◽  
pp. 651-658
Author(s):  
Samuel Gross ◽  
Vicki Keefer ◽  
Jerome Liebman

The mean platelet count in 102 children with congenital cyanotic heart disease was found to be 235,000/mm3 ± 1 S.D. of 85,000/mm3 as compared to 260,000 ± 70,000 in an age-matched group of normal children. In the cyanotic children, whose mean oxygen saturation was &gt;80%, the mean platelet level was 315,000/mm3 as compared to 185,000/mm3 in the &lt;60% oxygen saturation group. The platelet means were inversely related to the mean hemoglobins, such that the mean hemoglobin in the &gt;80% oxygen saturation group was 13.2 gm/100 ml as compared to 17.2 in the &lt;60% group. The age of the patient, and thus the duration of the disease, was also related to the platelet levels with significantly higher platelet counts in the children under 3 years of age. The mean age of the patients with platelet counts below 100,000/mm3 was 4.7 years as compared to a mean age of 2.8 years in the patients with platelet levels greater than 400,000/mm3. In addition, the older children had the highest hemoglobins and the lowest oxygen saturations. The inverse was true for the thrombocytotic individuals. The level of bound iron was not related to the platelet levels; nor were there any associated marrow or suspected capillary changes. It appears that the initial response to oxygen undersaturation is a platelet stimulation which subsequently results in underproduction as oxygen desaturation persists and worsens.


2019 ◽  
Vol 11 (1) ◽  
pp. 38
Author(s):  
Iwan Dwi Cahyono ◽  
Hari Hendriarto Satoto ◽  
Shazita Adiba Martyarini

Background: Tetralogy of fallot is one of the congenital cyanotic heart disease that is often found in children. The disorder has four features, a ventricular septal defect (VSD), aortic overriding, infundibulary stenotic, and hypertrophy right ventricular. Like other congenital heart disease, tetralogy of fallot sometimes related to fatal complications, such as bacterial endocarditis which was related to dental infections. Anesthetic management in tetralogy of Fallot is often described in patients with known cardiac disease. Perioperative considerations include preoperative preparation for surgery, intraoperative anesthetic management, and common postoperative issues in the intensive care unit.Case: A three-year-old boy had history of Tetralogy of Fallot. He has many severe early childhood caries. From the physical examination, many severe caries and roots gangrene was  found in both jaws. He was planned to get teeth extraction under general  anesthesia.Discussion: Tetralogy of fallot (TOF) is a congenital cyanotic heart disease that is often found in children, approximately around of 7–10% from overall congenital heart disease in children. Children with TOF have an increased risk of bacterial endocarditis. Invasive procedure was performed under general anesthesia. Patient was successfully operated under general anesthesia.Conclusion: Tetralogy of Fallot is a congenital cyanogenic heart disease that is a challenge for anesthetist. General anesthesia is the best suitable anesthetic technique in instable patient.


2017 ◽  
Vol 24 (3) ◽  
pp. 224-229 ◽  
Author(s):  
Christopher A Rouse ◽  
Brandon T Woods ◽  
C Becket Mahnke

Introduction Tele-echocardiography can ensure prompt diagnosis and prevent the unnecessary transport of infants without critical congenital heart disease, particularly at isolated locations lacking access to tertiary care medical centers. Methods We retrospectively reviewed all infants who underwent tele-echocardiography at a remote 16-bed level IIIB NICU from June 2005 to March 2014. Tele-echocardiograms were completed by cardiac sonographers in Okinawa, Japan, and transmitted asynchronously for review by pediatric cardiologists in Hawaii. Results During the study period 100 infants received 192 tele-echocardiograms: 46% of infants had tele-echocardiograms completed for suspected patent ductus arteriosus, 28% for suspected congenital heart disease, 12% for possible congenital heart disease in the setting of likely pulmonary hypertension, and 10% for possible congenital heart disease in the setting of other congenital anomalies. Of these, 17 patients were aeromedically evacuated for cardiac reasons; 12 patients were transported to Hawaii, while five patients with complex heart disease were transported directly to the United States mainland for interventional cardiac capabilities not available in Hawaii. Discussion This study demonstrates the use of tele-echocardiography to guide treatment, reduce long and potentially risky trans-Pacific transports, and triage transports to destination centers with the most appropriate cardiac capabilities.


2021 ◽  
Author(s):  
Kaori Hayashi ◽  
Akinori Hashiguchi ◽  
Masako Ikemiyagi ◽  
Hirobumi Tokuyama ◽  
Shu Wakino ◽  
...  

2021 ◽  
pp. 1-5
Author(s):  
Bhanu Jayanand Sudhir ◽  
Sanjay Honavalli Murali ◽  
Jaypalsinh Gohil ◽  
Rajalakshmi Poyuran ◽  
Manikantan Sethuraman ◽  
...  

Noninfectious cerebral aneurysms are rare in patients with congenital cyanotic heart disease. We present a patient with DiGeorge/velocardiofacial syndrome with a complex congenital cyanotic heart disease with a ruptured anterior communicating artery aneurysm. The 10-year-old child was managed by surgical clipping of the aneurysm. Surgical challenges included prominent veins in the Sylvian fissure, difficulty in differentiating arterial and venous bleed, and anesthetic risks. The patient recovered without any neurological deficits. This is the first report of a patient with 22q11.2 deletion syndrome, with a noninfectious cerebral aneurysm.


2016 ◽  
pp. bcr2015213615
Author(s):  
Francisco Abecasis ◽  
Inês Marques ◽  
Celeste Bento ◽  
Anabela Ferrão

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