Introduction to heart-ear syndrome
Cardio-auditory syndrome (also known as cardiac-deafness syndrome, QT interval syndrome) (Q-Tintervalsyndrome). There are three major characteristics: QT interval prolongation, syncope episodes and deafness, accompanied by congenital deafness, also known as Jervell-Lange-Neilsen syndrome, is autosomal recessive, and no deafness is also called Romano-Ward syndrome, which is often Chromosome dominant inheritance.basic knowledge
The proportion of illness: the incidence rate is about 0.0005%-0.0008%, more common in middle-aged and elderly people.
Susceptible people: no special people
Mode of infection: non-infectious
Complications: sudden death, ventricular premature contraction
Cause of heart-ear syndrome
(1) Causes of the disease
The etiology of this disease is still unknown. It is generally considered to be related to sympathetic dysfunction. Increasing sympathetic excitation, such as emotional excitement and physical activity, can induce symptoms.
In most cases, the tension between the sympathetic nerves on the left and right sides of the heart is unbalanced, and the right sympathetic tone is lower than the left side. According to the above inference, the clinical application of blockers and left cervical thoracic sympathectomy Satisfactory curative effect, pathological study found that sinus node, atrioventricular node, His bundle and ventricular myocardium have focal neuritis and neurodegeneration, ventricular myocardium neuropathy for ventricular repolarization abnormalities and QT interval Important relationship, and proposed that this neuropathy is caused by a chronic viral infection or some non-infectious degeneration. Other hypotheses still have myocardial metabolism, such as congenital deficiency of an enzyme in the myocardium and abnormal ventricular conduction system.
Heart-ear syndrome prevention
The cause is still unclear. With reference to the prevention of congenital diseases, preventive measures should be carried out from pre-pregnancy to prenatal:
Pre-marital medical examination plays an active role in preventing birth defects. The size of the effect depends on the examination items and contents, including serological examination (such as hepatitis B virus, treponema pallidum, HIV) and reproductive system examination (such as screening for cervical inflammation). General medical examinations (such as blood pressure, electrocardiogram) and asking about the family history of the disease, personal medical history, etc., do a good job in genetic disease counseling.
Pregnant women should avoid harmful factors as far as possible, including away from smoke, alcohol, drugs, radiation, pesticides, noise, volatile harmful gases, toxic and harmful heavy metals, etc. In the process of antenatal care during pregnancy, systematic screening of birth defects is required, including Regular ultrasound examination, serological screening, etc., if necessary, a chromosome examination.
Once an abnormal result occurs, it is necessary to determine whether to terminate the pregnancy; the safety of the fetus in the uterus; whether there is sequelae after birth, whether it can be treated, how to prognose, etc., and take practical measures for diagnosis and treatment.
The prenatal diagnostic techniques used are:
1 amniocytes culture and related biochemical examination (amniotic puncturing time is 16 to 20 weeks of pregnancy is appropriate).
2 pregnant women blood and amniotic fluid alpha fetoprotein determination.
3 ultrasound imaging (applicable in about 4 months of pregnancy).
4X-ray examination (after 5 months of pregnancy) is beneficial for the diagnosis of fetal skeletal deformities.
The sex chromatin of 5 villous cells was measured (40 to 70 days after conception), and the sex of the fetus was predicted to help diagnose the X-linked genetic disease.
6 application gene linkage analysis.
7 fetal mirror examination.
Through the application of the above technology, the birth of a fetus with severe genetic diseases and congenital malformations is prevented.
Heart-to-ear syndrome complications Complications, sudden death, ventricular contraction
Can cause sudden death, visible frequent premature ventricular contractions, ventricular tachycardia, ventricular fibrillation.
Symptoms of heart-ear syndrome Common symptoms Nausea arrhythmia Heart palpitations Hair loss vertigo QT interval prolonged convulsion chest tightness drowsiness
Arrhythmia is the main cause of syncope and sudden death. The main symptoms are short-term syncope episodes, often induced by excitement, anger, anxiety, excessive fatigue, etc. Some cases have chest tightness, palpitations, olfactory or somatosensory abnormalities before the onset, followed by Dizziness, syncope, lasting for a few seconds, more than ten seconds or more, and can show pale, sweating, cyanosis, body convulsions, incontinence and other symptoms, even sudden death, several minutes after the onset of disorientation, and some nausea, Vomiting, headache and general malaise, fatigue or lethargy, often misdiagnosed as epilepsy or rickets, mild episodes of unconscious loss, can feel temporary palpitations, chest swelling, blurred vision or dizziness, the number of episodes is uncertain, sometimes several times a day, one A few times a year, even 1 or 2 times in a lifetime, the first episode is more common in infants or young children, but can also be delayed until adolescents or later, especially those without deafness.
General routine inspections have no obvious abnormal results.
1. Electrocardiogram: characterized by prolongation of QT interval, T wave is wide, notch, high-point, two-way or inverted, and more with U wave fusion, the same patient in different periods or short-term QT interval and T wave changes very much Large, QT interval is more prolonged during emotional or exercise, and there is a T wave size alternation phenomenon, especially before fainting. Visible ventricular premature contraction or ventricular tachycardia can be seen before or after syncope. , ventricular fibrillation, occasional cardiac arrest, QT interval prolonged, so that the ventricular vulnerable period is extended and moved back, resulting in ventricular premature contraction is easy to fall in the vulnerable period and cause ventricular fibrillation, it is the main cause of syncope, sudden death The reason is that the QT interval tends to decrease with age, and the electrocardiogram is often accompanied by sinus bradycardia.
2. Hearing test: Some can find hearing loss.
Diagnosis and diagnosis of heart-ear syndrome
The diagnosis of typical cases is not difficult. For example, the QT interval is prolonged and the T wave changes are not obvious. The electrocardiogram can be checked after exercise. If the QT interval is prolonged, the T wave size is alternated, and the disease can still be diagnosed.
The syncope of QT prolongers was not found in time, and the patients were identified with epilepsy and rickets. EEG examination was helpful for the identification of epilepsy. Exercise tests were performed. QT prolongation of ECG should be guarded against this disease.