PMC9470943_01
Patient's ECG on admission.
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F1
A 37-year-old male was admitted to our hospital due to a complaint of "a sudden chest pain for 2 h." The ECG test indicated an acute anterior ST-segment elevation myocardial infarction (STEMI) (Figure 1).
Physical examination findings. (A) Thin skin, increased skin elasticity, and obviously exposed subcutaneous veins of the chest wall (yellow circle). (B) Visible veins and subcutaneous ecchymosis (red circle) on the forearm. (C) Visible subcutaneous ecchymosis on the lower limbs (red circles). (D,E) Abnormally increased mobility of the bilateral little finger MCP joints. (F) Genetic testing: A heterozygous mutation of c.1347 + 1G > A in the COL3A1 gene was found and verified using PCR and Sanger sequencing, which led to a protein sequence splicing mutation.
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F2
The physical examination showed that the patient had slightly protruding eyes, thin skin, increased skin elasticity, obviously exposed chest and forearm subcutaneous veins, visible subcutaneous ecchymosis on the forearm and lower limbs (Figures 2A-C), and an abnormally increased mobility of the little bilateral finger metacarpophalangeal (MCP) joints (Figures 2D,E). A heterozygous mutation of c.1347 + 1G > A in the COL3A1 gene was also discovered and verified using PCR and Sanger sequencing, which indicated a protein sequence splicing mutation (Figure 2F).
Imaging findings. (A,B) Coronary angiography (CAG). The white arrow shows a type 1 dissection at the proximal segments of LAD. The yellow arrow indicated a type 2 dissection at the middle segments of the LAD and the diagonal branch. (C) The patient showed progression of coronary artery dissection during the CAG. The red arrow demonstrates that the dissection involved the LM and the yellow arrow indicates a type 4 dissection of the LCX. (D) After revascularization. The white arrow shows the TIMI flow grade 3 at the LM and LAD. The yellow arrow indicates the TIMI flow grade 3 at the LCX. (E) IVUS showed a massive IMH from the media external to the LAD stent; the white arrow indicated the stent struts. (F) The true lumen (TL) of the median LCX segment was severely deformed by the dissection and the massive media layer hematoma. GW, guidewire. (G,H) The chest CT revealed a stent in the left anterior descending coronary artery and hemopericardium. (I,J) The abdominal and pelvic CT showed intestinal wall swelling, hematocele, retroperitoneal hematocele, and an iliac vascular dissection (see section "Supplementary material").
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F3
The mentioned findings prompted an emergency selective CAG via the right radial artery, which indicated a dissection located between the proximal and middle segments of the left anterior descending (LAD) branch (Figures 3A,B). The results indicated a progression of the coronary artery dissection involving the left main (LM) trunk and the left circumflex branch (LCX), which caused an LCX occlusion of the proximal segment (type 4 dissection of the LCX) (Figure 3C) with the Thrombolysis in Myocardial Infarction (TIMI) flow grade 0 during CAG. It showed a massive intramural hematoma (IMH) from the media external to the LAD stent, with the true vascular lumen of the median LCX segment being severely deformed by the dissection and the massive media layer hematoma (Figures 3E,F). TIMI flow grade 3 (Figure 3D). The patient was transferred to the cardiac care unit for treatment following the procedure. The chest CT revealed a stent in the left anterior descending coronary artery and hemopericardium (Figures 3G,H). Meanwhile, the abdominal and pelvic CT showed intestinal wall swelling, hematocele, retroperitoneal hematocele, and an iliac vascular dissection (Figures 3I,J).