Case 1
A 13-year-old female patient (height:150 cm; weight: 42 kg) was diagnosed with functional SV combined with transposition of the great arteries (TGA) with a right position of the aorta based on ultrasound findings at the age of 3, but did not receive any treatment. In 2017, she came to our hospital due to numbness in both lower limbs and dyskinesia. Thoracic magnetic resonance imaging (MRI) showed a space-occupying lesion in the spinal canal. A bone biopsy was performed and revealed cavernous hemangioma. This patient underwent a tumor resection at another hospital and returned to our hospital for radiotherapy 5 months after the operation.Physical examination at our hospital showed a temperature of 36.5℃, a pulse of 80 beats/min, no difference in the pulse and heart rate (HR), a respiratory rate of 18 breaths/min, and a blood pressure of 120/80 mmHg.Other pertinent examinations showed no signs of anemia and she had no history of hemoptysis, syncope, or long-term high fever. The patient exhibited normal development, moderate nutrition. Her New York Heart Association functional class was II. Echocardiography revealed the following: the heart was located in the left thoracic cavity, the atrium was in the normal position, the left ventricular cavity was the main cavity, and the right ventricular cavity was the residual cavity. The pulmonary artery (PA) located on the posterior side and arose from the left cavity. The aorta was moderately developed, located on the anterior side and arose from the right cavity.On Echocardiography, the upper interventricular septum was interrupted by 22.3 mm(Fig. 1). Color Doppler flow imaging (CDFI) revealed a two-way low-resistance shunt in the ventricular septal defect. The patient was diagnosed with the following diseases based on the echocardiography results: congenital heart disease, functional SV, TGA, mild PS, left ventricular hypertrophy, and left atrial enlargement. Further examination was recommended. No tumor metastasis was found after radiotherapy at our hospital and the prognosis was appreciable.
Case 2
A 41-year-old female patient (height: 163 cm, weight: 50 kg) was admitted to our hospital due to chest pain, chest tightness, and palpitations lasting for 3 days. She presented with cyanosis of the mouth and limb immediately after birth, with obvious cyanosis after crying but without syncope or squatting signs. She was diagnosed with a congenital heart disease at the local hospital but was not treated. Her growth and development were not affected and daily activities were only slightly limited. After catching a cold 3 days earlier, she experienced chest tightness and shortness of breathafter general physical activities. After symptomatic treatment at the local hospital, she was admitted to our hospital due to a lack of obvious symptom relief.
Physical examination at our hospital revealed a temperature of 36.6℃, a pulse of 62 beats/min, a respiratory rate of 20 breaths/min, a blood pressure of 110/60 mmHg,clear consciousness, no yellowing of the skin and sclera, and cyanosis of the mouth.Her heart rate was 62 beats/min, the heart rhythm was uniform. Her New York Heart Association functional class was Ⅲ. Echocardiography showed dextrocardia as well as a heart with a functional single atrium and SV, ventricular hypertrophy, and a common atrioventricular valve. The atrial and ventricular septa were not explored but were clearly defined. Two large arteries, the PA and the aorta, were concomitant, both starting from the functional SV. The PA was located on the anterior side and the aorta was located on the posterior side. No obvious abnormalities were found in the aortic valve. CDFI revealed severe reflux in the common atrioventricular valve in the systolic stage, the neck of the valve orifice reflux was 13.4 mm, the flow rate was 4.0 m/s, and the pressure gradient (PG) was 63 mmHg(Fig. 2).Under three-dimensional ultrasonic examination, the atrioventricular valves appeared to consist of four valves. The echocardiographic diagnosis for this patient was congenital heart disease complex deformity, dextrocardia, single atrium, SV, PS, and severe common atrioventricular valve insufficiency. The patient was discharged after 5 days of anti-infection and symptomatic treatments. After discharge, we continued to follow up with the patient by telephone and outpatient visits.
Case 3
A 13-year-old female patient (height: 137 cm; weight: 40 kg) came to our hospital due to palpitations and chest tightness lasting for 2 days. She was diagnosed with a complex congenital heart disease at the age of 6 months but was not treated. Physical examination on admission showed a body temperature of 36.4℃, a pulse of 57 beats/min, a respiratory rate of 21 breaths/min, and a blood pressure of 105/70 mmHg. Her New York Heart Association functional class was II. Echocardiography showed the following: the heart was located in the left thoracic cavity, the atrium was in the normal position, the PA and the aorta were concomitant, and the PA was located in front of the aorta, both starting from a functional SV. The right atrium was enlarged, no atrioventricular valve was found in the left ventricular cavity parallel to the right atrium, the echo of the interventricular atrium was interrupted by 10.2 mm, and the internal diameter of the SV was 65 mm. CDFI showed a shunt in the atrial septal defect with a flow rate of 2.6 m/s and a PG of 29 mmHg.Therewere severe reflux in the atrioventricular valve in the systolic stage, the regurgitation area of the atrioventricularvalve orifice was 3.7 cm 2, the flow rate was 5.4 cm/s, and the PG was 117 mmHg (Fig. 3).The length of the pulmonary valve regurgitation beam was 22.8 mm, the flow rate was 3.5 m/s, the PG was 50 mmHg, and the average PA pressure was estimated to be 55 mmHg. The echocardiographic diagnosis was congenital heart disease (SV,atrial septal defect), mild atrioventricular valve insufficiency, mild pulmonary valve regurgitation, and pulmonary hypertension (PHT). The patient was discharged after 3 days of symptomatic treatment.
Case 4
A 39-year-old woman (height: 162 cm; weight: 50 kg) came to our hospital for chest pain and chest tightness lasting for 4 days. She was diagnosed with a congenital heart disease at birth but was not treated. Physical examination on admission showed a temperature of 37.0℃, a pulse of 60 beats /min, a respiratory rate of 22 breaths/min, and a blood pressure of 110/80 mmHg. Her New York Heart Association functional class was II. Echocardiography showed the following: the heart was located in the left thoracic cavity, the atrium was in the normal position, the positions of the PA and the aorta were abnormal, the aorta had widened and moved forward, and both the heart and PA started from the functional SV. There was noventricular septum,the internal diameter of the SV was 58.2 mm (Fig. 4). CDFI revealed that the regurgitation area of the tricuspid valve orifice was 3.1 cm2, the flow rate was 4.1m/s, and the PG was 66 mmHg.The echocardiographic diagnosis for this patient was congenital heart disease (SV, left ventricular type, PHT, and mild tricuspid regurgitation. The patient was discharged after 4 days of symptomatic treatment.