Congenital Heart Disease in Patients With Cleft Lip/Palate and Its Impact on Cleft Management

Objective: To evaluate characteristics of congenital heart disease (CHD) in patients with cleft lip and/or palate (CL/P) and assess potential associations with cleft outcomes. Design: Retrospective review of all patients with CL/P who underwent primary cleft treatment from 2009 to 2015. Setting: Children’s Hospital Los Angeles, a tertiary hospital. Patients: Exclusion criteria included microform cleft lip diagnosis, international patients, and patients presenting for secondary repair or revision after primary repair at another institution. Main Outcomes Measured: Patient demographics, prenatal and birth characteristics, CL/P characteristics, syndromic status, postoperative complications, and other outcomes were analyzed relative to CHD diagnoses and management. Patients with CL/P with (+CHD) were compared to those without (−CHD) CHD using χ2 tests and analysis of variance. Results: Among 575 patients with CL/P, 83 (14.4%) had CHD. Congenital heart disease rates were significantly higher in patients with cleft palate (CP) compared to other cleft types (χ2, P = .009). Eighty-one (97.6%) out of 83 +CHD patients were diagnosed prior to initial CL/P surgical assessment. Twenty-three (27.7%) +CHD patients required surgical repair of 10 cardiac anomalies prior to cleft care. Congenital heart disease was associated with delayed CP repair and increased rates of fistula in isolated patients with CP. Conclusions: Congenital heart disease is known to be more prevalent in patients with CL/P. These data suggest the condition is particularly increased in patients with CP. Severe forms of CHD are diagnosed and treated prior to cleft care however postoperative fistula may be more common in patients with CHD. Therefore, careful attention is required for patient optimization and palatal flap dissection in patients with coexisting CHD and CL/P.

Download Full-text

The 22q11.2 Microdeletion in Pediatric Patients with Cleft Lip, Palate, or Both and Congenital Heart Disease: A Systematic Review

Journal of Pediatric Genetics ◽

10.1055/s-0039-1698804 ◽

2019 ◽

Vol 09 (01) ◽

pp. 001-008

Author(s):

Diana Cárdenas-Nieto ◽

Maribel Forero-Castro ◽

Clara Esteban-Pérez ◽

Julio Martínez-Lozano ◽

Ignacio Briceño-Balcázar

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Early Diagnosis ◽

Congenital Heart ◽

Cleft Lip ◽

Pediatric Patients ◽

Multidisciplinary Treatment ◽

22Q11.2 Deletion Syndrome ◽

Deletion Syndrome ◽

Cleft Lip Palate

AbstractThe 22q11.2 deletion syndrome (22q11.2DS) is present in approximately 5 to 8% of patients with cleft lip, palate, or both (CL/P) and 75 to 80% of patients with congenital heart disease (CHD). In a literature review, we consider this association of 22q11.2DS in pediatric patients with CL/P and CHD. Early diagnosis of 22q11.2DS in pediatric patients with CL/P and CHD helps to optimize a multidisciplinary treatment approach for 22q11DS. Early diagnosis, thereby, can improve quality of life for these patients and awareness of other potential clinical implications that may require attention throughout the patient's life.

Download Full-text

Down's Syndrome with Congenital Heart Disease: Analysis of Cases Over Two Years in a Non-Invasive Laboratory of a Tertiary Hospital

Cardiovascular Journal ◽

10.3329/cardio.v2i2.6637 ◽

1970 ◽

Vol 2 (2) ◽

pp. 184-187

Author(s):

NN Fatema

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Doppler Echocardiography ◽

Congenital Heart ◽

Down's Syndrome ◽

Down’S Syndrome ◽

Tertiary Hospital ◽

Study Group ◽

Non Invasive ◽

Study Results

Background: Trisomy 21 or Down's Syndrome is the most frequent chromosomal aberration affecting live birth infants with an incidence of 1 in 660 live births. This syndrome is often associated with congenital cardiac lesions, Incidence of which is 40-60 percent. This study was conducted to see the frequency of Down’s syndrome cases and pattern of heart diseases they have in one of the busy non-invasive pediatric cardiac laboratory of the country. Methods: It was a retrospective study conducted in the non-invasive pediatric cardiac laboratory and pediatric cardiac outpatient clinic of a tertiary hospital over a period of two years (November 2007 to October 2009). The entire patients who had Down's Syndrome and had Doppler echocardiography were included in the study. Results: Out of total six thousand and fifty echocardiography, Down's Syndrome case was 205 (3.38%). Out of 205 cases, 185 cases were followed up in pediatric cardiac out patient clinic. Twenty cases had not reported in the out patient clinic. Seventeen of those patients had normal cardiac anatomy in Doppler echocardiography. Male were 43.90% and female were 56.09% amongst study group. Most of the patients are young infant (47.32%). Only 2.44% are in more than 10 years age group. Murmur was audible in 86.49% cases in study group and developmental delay was present in 100% of the cases. Doppler Echocardiography was found as most sensitive and specific investigation for detecting congenital heart disease. A-V canal defect was the commonest association (15.60%). Congenital heart disease was not detected in 8.29% cases. Surgical treatment was advised in 52.19% cases, Device closure was advised in 16.59% cases, medical management was advised in 21.46% cases. Conclusion: Down's syndrome is a very common chromosomal anomaly in our country. Incidence of this syndrome is increasing as number of working women, late marriage and elderly mother increasing. So, multidisciplinary approach for managing this disease should be adopted immediately. Keywords: Down's syndrome; Congenital heart disease. DOI: 10.3329/cardio.v2i2.6637Cardiovasc. j. 2010; 2(2) : 184-187

Download Full-text

Asymmetric Crying Facies: Which Is the `Right-Wrong' Side?

PEDIATRICS ◽

10.1542/peds.71.1.144a ◽

1983 ◽

Vol 71 (1) ◽

pp. 144-145

Author(s):

KARL-GEORG EVERS ◽

PETER GRONECK

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Soft Palate ◽

Congenital Heart ◽

Cleft Lip ◽

Congenital Defects ◽

Wrong Side ◽

Organ Systems ◽

Depressor Muscle ◽

The Right

To the Editor.— Congenital asymmetric crying facies is generally considered to be due to unilateral agenesis or hypoplasia of the anguli oris depressor muscle (HAODM). Electromyographic (EMG) examinations of the affected sides have revealed absent spontaneous activity or diminished motor unit activity.1-3 Association of HAODM syndrome with congenital heart disease, the "cardiofacial syndrome," has been described.4 Major defects of other organ systems and minor congenital defects may be associated with asymmetric crying facies as well.5 Monreal6 reported five patients with asymmetric congenital crying facies syndroms who besides this anomaly displayed juxtaoral defects, egm atresia of one side of the jaw and soft palate, cleft lip, hypoplasis of mandible and ear.

Download Full-text

Experiences of Mothers Facing the Prognosis of Their Children with Complex Congenital Heart Disease

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph17197134 ◽

2020 ◽

Vol 17 (19) ◽

pp. 7134

Author(s):

Sunhee Lee ◽

Jeong-Ah Ahn

Keyword(s):

Congenital Heart Disease ◽

Content Analysis ◽

Heart Disease ◽

Qualitative Study ◽

Congenital Heart ◽

Tertiary Hospital ◽

Complex Congenital Heart Disease ◽

Face To Face ◽

Psychological Difficulties ◽

Expressed Emotions

Mothers of children with complex congenital heart disease face unique challenges and emotional burdens, while their children go through physical and psychological difficulties during disease progression. In this study, we aimed to explore the in-depth experiences and feelings of mothers facing the prognosis of their children with complex congenital heart disease that was surgically corrected. This is a descriptive qualitative study. We conducted semi-structured, face-to-face interviews with 12 mothers of children with complex congenital heart disease at a tertiary hospital in Seoul, Korea. The interview data were analyzed by content analysis. Participants were mothers aged between 40–58 years whose children were diagnosed with complex congenital heart disease which was surgically corrected. Based on the content analysis, the mothers’ experiences and feelings were categorized as immense suffering and adapting to a new life. Under the main categories, the concepts included feeling of abandonment, anxiety with potentially losing their children, having hope, seeking reassurance, being encouraged, and trying to embrace the situation. Mothers who cared for their children with complex congenital heart disease expressed emotions that changed sequentially alongside physical and psychosocial changes in the children. The results of this study are valuable for understanding the experiences and emotions of mothers facing the prognosis of their children with complex congenital heart disease in order to aid in the development of programs that support these mothers.

Download Full-text